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Charous SJ, Yuhan B, Stenson KM, Talati V, McMullen P. Dedifferentiation of a Chondrosarcoma of the Larynx: A Case Report. Head Neck 2025; 47:E58-E63. [PMID: 39744978 DOI: 10.1002/hed.28004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2024] [Revised: 10/15/2024] [Accepted: 11/06/2024] [Indexed: 04/30/2025] Open
Abstract
BACKGROUND Chondrosarcomas of the larynx, relatively rare tumors with low grade pathology in approximately 95% of cases, can most often be managed with conservation laryngeal procedures. Dedifferentiated chondrosarcomas are much more rare and aggressive requiring more aggressive surgical extirpation. METHODS A patient underwent three debulking procedures for a laryngeal chondrosarcoma Grade I/II histologically over a 2.5 year period of time during which slow growth was closely monitored. He then presented with relatively rapid onset of hoarseness and shortness of breath. Fiberoptic laryngoscopy demonstrated new bilateral vocal fold fixation with near occlusion of his subglottis by soft tissue mass. RESULTS A total laryngectomy was performed that pathologically demonstrated a Grade III dedifferentiated chondrosarcoma. Shortly thereafter, the patient developed regional metastases, then distant metastases and then succumbed to the disease. CONCLUSION This first case of a Grade III dedifferentiating chondrosarcoma arising from closely monitored and multiple debulked previously Grade I/II tumor demonstrates the potential change in aggressiveness in low-grade chondrosarcomas of the larynx. Sudden increase in growth along with a soft tissue component to the tumor based on exam and/or imaging should heighten suspicion for dedifferentiation of the tumor and necessitates a more aggressive surgical resection. However, the chance of cure once dedifferentiation occurs is drastically reduced.
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Affiliation(s)
- Steven J Charous
- Department of Otolaryngology - Head and Neck Surgery, Loyola University Medical Center, Maywood, Illinois, USA
| | - Brian Yuhan
- Department of Otolaryngology - Head and Neck Surgery, Loyola University Medical Center, Maywood, Illinois, USA
| | - Kerstin M Stenson
- Department of Otolaryngology - Head and Neck Surgery, Rush University Medical Center, Chicago, Illinois, USA
| | - Vidit Talati
- Department of Otolaryngology - Head and Neck Surgery, Rush University Medical Center, Chicago, Illinois, USA
| | - Phillip McMullen
- Department of Otolaryngology - Head and Neck Surgery, Loyola University Medical Center, Maywood, Illinois, USA
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Gasparro MA, Lee L, Vijayakumar G, Gusho CA, Colman MW, Gitelis S, Miller I, Blank AT. Rare Dedifferentiated Periosteal Chondrosarcoma with 11-Year Metastatic Relapse: A Case Report. Int J Surg Pathol 2024; 32:201-205. [PMID: 37143346 DOI: 10.1177/10668969231173379] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/06/2023]
Abstract
A 50-year-old man presented for evaluation of a periscapular mass. Biopsy suggested a low-grade chondrosarcoma; however, the resected specimen revealed a grade 2 chondrosarcoma with a low-grade dedifferentiated mesenchymal component. The mass multiply recurred as chondrosarcoma without a dedifferentiated component before the patient developed lung metastases of chondrosarcoma without a dedifferentiated component 11 years after the initial diagnosis and died of disease. This is one of the first reported cases of a dedifferentiated chondrosarcoma with low-grade dedifferentiated component. While overall prognosis may be better than in typical dedifferentiated chondrosarcoma, this tumor demonstrated numerous local recurrences as well as metastasis.
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Affiliation(s)
- Matthew A Gasparro
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Linus Lee
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Gayathri Vijayakumar
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Charles A Gusho
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Matthew W Colman
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Steven Gitelis
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
| | - Ira Miller
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
- Department of Pathology, Rush University Medical Center, Chicago, IL, USA
| | - Alan T Blank
- Department of Orthopedic Surgery, Division of Oncology, Rush University Medical Center, Chicago, IL, USA
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3
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Hanona PF, Ezekwudo D, Anderson J, Jaiyesimi I. Chondrosarcoma With Pulmonary Metastatic Calcifications: A Case Report and Review of the Literature. Cureus 2024; 16:e53234. [PMID: 38425591 PMCID: PMC10903573 DOI: 10.7759/cureus.53234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/30/2024] [Indexed: 03/02/2024] Open
Abstract
A chondrosarcoma with pulmonary metastatic calcifications is a rarely reported phenomenon. This report discusses chondrosarcomas and their clinical features, diagnosis, and treatment, using as an example the case of a 55-year-old female with a right pelvic chondrosarcoma that developed over 10 years. In the last two years, the patient had increasing pulmonary findings, including pulmonary nodules, ground glass opacities, and likely pulmonary metastatic calcifications. The objective of this report is to explore chondrosarcomas and their pattern of metastatic presentation, with the hope of improving recognition of the disease and streamlining treatment.
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Affiliation(s)
- Paul F Hanona
- Hematology and Oncology, Corewell Health William Beaumont University Hospital, Royal Oak, USA
| | - Daniel Ezekwudo
- Hematology and Oncology, Corewell Health William Beaumont University Hospital, Royal Oak, USA
| | - Joseph Anderson
- Hematology and Oncology, Corewell Health William Beaumont University Hospital, Royal Oak, USA
| | - Ishmael Jaiyesimi
- Hematology and Oncology, Corewell Health William Beaumont University Hospital, Royal Oak, USA
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El Beaino M, Hoda ST, Eldeib AJ, Masrouha K. Dedifferentiated Chondrosarcoma: Diagnostic Controversies and Emerging Therapeutic Targets. Curr Oncol Rep 2023; 25:1117-1126. [PMID: 37603119 DOI: 10.1007/s11912-023-01441-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/10/2023] [Indexed: 08/22/2023]
Abstract
PURPOSE OF REVIEW The pathogenesis of dedifferentiated chondrosarcoma is controversial, and no genetic abnormality has consistently been identified in the disease. Focusing on the diagnostic challenges encountered in dedifferentiated chondrosarcoma, the following review aims at summarizing the tumor's active neoplastic pathways while highlighting therapeutic modalities that could potentially be explored to enhance patient survivorship. RECENT FINDINGS Owing to the challenging examination of small needle biopsy sampling as well as the disease's overlapping morphological and immunohistochemical features with other bone and soft-tissue sarcomas, the diagnosis of dedifferentiated chondrosarcoma can be problematic. While combined doxorubicin- and cisplatin-based regimens remain the first-line systemic chemotherapy in the disease, ~50% of tumors carry EXT1/2 or IDH1/2 mutations, advancing EXT or IDH inhibitors as potential alternative therapies, respectively. Despite systemic chemotherapy, dedifferentiated chondrosarcoma remains an aggressive tumor with dismal prognosis and limited survival. A multidisciplinary collaboration across multiple cancer centers is warranted to yield an accurate diagnosis, understand the disease's underlying pathogenesis, develop adequate treatment, and improve patient survivorship.
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Affiliation(s)
- Marc El Beaino
- Department of Orthopaedic Surgery and Rehabilitation Medicine, State University of New York, Downstate Health Sciences University, Brooklyn, NY, USA.
- School of Public Health, State University of New York, Downstate Health Sciences University, Brooklyn, NY, USA.
| | - Syed T Hoda
- Department of Surgical Pathology, New York University Langone Health, New York, NY, USA
| | - Ahmed J Eldeib
- Department of General Surgery, State University of New York, Downstate Health Sciences University, Brooklyn, NY, USA
| | - Karim Masrouha
- Department of Orthopaedic Surgery, New York University Langone Health, New York, NY, USA
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Zając W, Dróżdż J, Kisielewska W, Karwowska W, Dudzisz-Śledź M, Zając AE, Borkowska A, Szumera-Ciećkiewicz A, Szostakowski B, Rutkowski P, Czarnecka AM. Dedifferentiated Chondrosarcoma from Molecular Pathology to Current Treatment and Clinical Trials. Cancers (Basel) 2023; 15:3924. [PMID: 37568740 PMCID: PMC10417069 DOI: 10.3390/cancers15153924] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2023] [Revised: 07/27/2023] [Accepted: 07/28/2023] [Indexed: 08/13/2023] Open
Abstract
Dedifferentiated chondrosarcoma (DDCS) is a rare subtype of chondrosarcoma, a primary cartilaginous malignant neoplasm. It accounts for up to 1-2% of all chondrosarcomas and is generally associated with one of the poorest prognoses among all chondrosarcomas with the highest risk of metastasis. The 5-year survival rates range from 7% to 24%. DDCS may develop at any age, but the average presentation age is over 50. The most common locations are the femur, pelvis humerus, scapula, rib, and tibia. The standard treatment for localised disease is surgical resection. Most patients are diagnosed in unresectable and advanced stages, and chemotherapy for localised and metastatic dedifferentiated DDCS follows protocols used for osteosarcoma.
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Affiliation(s)
- Weronika Zając
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
- Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Julia Dróżdż
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
- Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Weronika Kisielewska
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
- Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Weronika Karwowska
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
- Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Monika Dudzisz-Śledź
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
| | - Agnieszka E. Zając
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
| | - Aneta Borkowska
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
| | - Anna Szumera-Ciećkiewicz
- Department of Pathology, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland;
| | - Bartłomiej Szostakowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
| | - Piotr Rutkowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
| | - Anna M. Czarnecka
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska Curie National Research Institute of Oncology, 02-781 Warsaw, Poland (M.D.-Ś.); (A.E.Z.); (A.B.); (B.S.); (P.R.)
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Abdullah L, Alsulaiman SS, Alsalhani OM, Alkheder A, Al-Sulaiman IA. A rare case of chondrosarcoma at the posterior costovertebral junction: a case report. Ann Med Surg (Lond) 2023; 85:3129-3132. [PMID: 37363554 PMCID: PMC10289765 DOI: 10.1097/ms9.0000000000000821] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2023] [Accepted: 05/01/2023] [Indexed: 06/28/2023] Open
Abstract
UNLABELLED Chodrosarcoma is the third most common primary malignancy of the bone, it is considered the most frequent malignancy of the chest wall. Chondrosarcoma at the costovertebral junction is rare and few cases are described in the literature. CASE PRESENTATION The authors present a case of a 47-year-old female with a complaint of chronic pain in the right posterior upper chest, dyspnea, and episodes of dizziness.The diagnosis of low-grade chondrosarcoma at the posterior costovertebral junction was made by video-assisted thoracic surgery and computed tomography.The surgical management included resection of the tumor and parts of adjacent ribs. DISCUSSION Chondrosarcoma is chemotherapy and radiotherapy-resistant. The total surgical excision of the tumor with sufficient margins is usually curative. CONCLUSION A follow-up is necessary because of the potential for recurrence.
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Affiliation(s)
| | | | | | - Ahmad Alkheder
- Otorhinolaryngology Department, Al-Mowassat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
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Kim JH, Lee SK. Classification of Chondrosarcoma: From Characteristic to Challenging Imaging Findings. Cancers (Basel) 2023; 15:cancers15061703. [PMID: 36980590 PMCID: PMC10046282 DOI: 10.3390/cancers15061703] [Citation(s) in RCA: 22] [Impact Index Per Article: 11.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2023] [Revised: 03/08/2023] [Accepted: 03/08/2023] [Indexed: 03/18/2023] Open
Abstract
Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone classifies chondrogenic bone tumors as benign, intermediate (locally aggressive), or malignant, and separates atypical cartilaginous tumors (ACTs) and chondrosarcoma grade 1 (CS1) as intermediate and malignant tumors. respectively. Furthermore, the classification categorizes chondrosarcomas (including ACT) into eight subtypes: central conventional (grade 1 vs. 2–3), secondary peripheral (grade 1 vs. 2–3), periosteal, dedifferentiated, mesenchymal, and clear cell chondrosarcoma. Most chondrosarcomas are the low-grade, primary central conventional type. The rarer subtypes include clear cell, mesenchymal, and dedifferentiated chondrosarcomas. Comprehensive analysis of the characteristic imaging findings can help differentiate various forms of chondrosarcomas. However, distinguishing low-grade chondrosarcomas from enchondromas or high-grade chondrosarcomas is radiologically and histopathologically challenging, even for experienced radiologists and pathologists.
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Affiliation(s)
- Jun-Ho Kim
- Department of Orthopaedic Surgery, Center for Joint Diseases, Kyung Hee University Hospital at Gangdong, Seoul 05278, Republic of Korea
| | - Seul Ki Lee
- Department of Radiology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea
- Correspondence:
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8
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Hung YP, Chebib I, Bredella MA, Berner EA, Taylor-Black Q, Choy E, Cote GM, Chen YL, MacDonald SM, Schwab JH, Raskin KA, Newman ET, Selig MK, Deshpande V, Hornick JL, Lozano-Calderón SA, Nielsen GP. Prognostic Significance of Percentage and Size of Dedifferentiation in Dedifferentiated Chondrosarcoma. Mod Pathol 2023; 36:100069. [PMID: 36788104 DOI: 10.1016/j.modpat.2022.100069] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2022] [Revised: 11/17/2022] [Accepted: 12/11/2022] [Indexed: 01/11/2023]
Abstract
Dedifferentiated chondrosarcoma is rare, aggressive, and microscopically bimorphic. How pathologic features such as the amounts of dedifferentiation affect prognosis remains unclear. We evaluated the percentages and sizes of dedifferentiation in a consecutive institutional series of dedifferentiated chondrosarcomas from 1999 to 2021. The statistical analysis included cox proportional hazard models and log-rank tests. Of the 67 patients (26 women, 41 men; age, 39 to >89 [median 61] years; 2 with Ollier disease), 58 presented de novo; 9 were identified with conventional chondrosarcomas 0.6-13.2 years (median, 5.5 years) prior. Pathologic fracture and distant metastases were noted in 27 and 7 patients at presentation. The tumors involved the femur (n = 27), pelvis (n = 22), humerus (n = 7), tibia (n = 4), scapula/ribs (n = 4), spine (n = 2), and clivus (n = 1). In the 56 resections, the tumors ranged in size from 3.5 to 46.0 cm (median, 11.5 cm) and contained 1%-99.5% (median, 70%) dedifferentiated components that ranged in size from 0.6 to 24.0 cm (median, 7.3 cm). No correlation was noted between total size and percentage of dedifferentiation. The dedifferentiated components were typically fibrosarcomatous or osteosarcomatous, whereas the associated cartilaginous components were predominantly grade 1-2, rarely enchondromas or grade 3. The entire cohort's median overall survival and progression-free survival were 11.8 and 5.4 months, respectively. In the resected cohort, although the total size was not prognostic, the percentage of dedifferentiation ≥20% and size of dedifferentiation >3.0 cm each predicted worse overall survival (9.9 vs 72.5 months; HR, 3.76; 95% CI, 1.27-11.14; P = .02; 8.7 vs 58.9 months; HR, 3.03; 95% CI, 1.21-7.57; P = .02, respectively) and progression-free survival (5.3 vs 62.1 months; HR, 3.05; 95% CI, 1.13-8.28; P = .03; 5.3 vs 56.6 months; HR, 2.50; 95% CI, 1.06-5.88; P = .04, respectively). In conclusion, both the percentages and sizes of dedifferentiation were better prognostic predictors than total tumor sizes in dedifferentiated chondrosarcomas, highlighting the utility of their pathologic evaluations.
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Affiliation(s)
- Yin P Hung
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts.
| | - Ivan Chebib
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts
| | - Miriam A Bredella
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Emily A Berner
- Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Quinn Taylor-Black
- Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Edwin Choy
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Division of Hematology Oncology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts
| | - Gregory M Cote
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Division of Hematology Oncology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts
| | - Yen-Lin Chen
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts
| | - Shannon M MacDonald
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts
| | - Joseph H Schwab
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Kevin A Raskin
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Erik T Newman
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Martin K Selig
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - Vikram Deshpande
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts
| | - Jason L Hornick
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts
| | - Santiago A Lozano-Calderón
- Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts; Department of Orthopedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
| | - G Petur Nielsen
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts
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Tanutit P, Pakdee W, Laohawiriyakamol T, Iamthanaporn K. Magnetic resonance imaging in differentiating between aggressive and non-aggressive bone tumors. Acta Radiol 2023; 64:625-637. [PMID: 35306878 DOI: 10.1177/02841851221082098] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
BACKGROUND While radiography remains essential in the initial evaluation of bone lesions, tissue biopsy or further imaging is often required to clarify indeterminate radiographic features. Magnetic resonance imaging (MRI) assists radiologists in evaluating lesions with indeterminate features as it has advantages in delineating tumorous tissues and bone marrow. PURPOSE To evaluate the association factors of MRI for bony aggressiveness. MATERIAL AND METHODS A retrospective analysis of 226 MRI examinations from patients diagnosed with bone tumors in a tertiary hospital during 2008-2018 was performed. All the MR images were interpreted by musculoskeletal radiologists without diagnostic information. The bony lesions were categorized into aggressive and non-aggressive groups using tumor margin, cortical changes, periosteal reaction, joint extension, extraosseous soft tissue involvement, tumor homogeneity, and enhancement pattern from the MR images. Univariable and multivariable analysis were applied for each feature on the MRI scans. In addition, sensitivity and specificity were calculated for MRI diagnoses of aggressive bone lesions. RESULTS In total, 180 aggressive and 46 non-aggressive bone lesions were examined on MRI. The sensitivity and specificity of MRI for differentiating between aggressive and non-aggressive bone lesions were 98.89% and 50%, respectively. Ill-defined margin, cortical break, cortical signal changes, sunburst and Codman's triangle periosteal reaction, joint extension, and tumoral and heterogeneous enhancement could be predictive signs for aggressive bone lesions. CONCLUSION MRI can be a valuable tool to assist in distinguishing aggressive from non-aggressive bone lesions. In cases of indeterminate radiographic features, MRI could be used as an additional imaging to improve diagnostic accuracy and could reduce unnecessary invasive procedures.
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Affiliation(s)
- Pramot Tanutit
- Department of Radiology, Faculty of Medicine, 26686Prince of Songkla University, Songkhla, Thailand
| | - Wisitsak Pakdee
- Department of Radiology, Faculty of Medicine, 26686Prince of Songkla University, Songkhla, Thailand
| | | | - Khanin Iamthanaporn
- Department of Orthopaedic Surgery and Physical Medicine, Faculty of Medicine, 26686Prince of Songkla University, Songkhla, Thailand
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Gersey ZC, Zenonos GA, Gardner PA. Malignant Brain and Spinal Tumors Originating from Bone or Cartilage. ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY 2023; 1405:477-506. [PMID: 37452950 DOI: 10.1007/978-3-031-23705-8_18] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 07/18/2023]
Abstract
Malignant bone tumors affecting the brain and spine are a rare and exceedingly difficult-to-treat group of diseases. Most commonly consisting of chordoma and chondrosarcoma, these tumors also include giant-cell tumors and osteosarcomas. This chapter will cover the background, epidemiology, genetics, molecular biology, histopathology, radiographic features, clinical manifestations, therapeutic approaches, and clinical management of each entity.
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Affiliation(s)
- Zachary C Gersey
- Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Georgios A Zenonos
- Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Paul A Gardner
- UPMC Center for Cranial Base Surgery, 200 Lothrop Street, PUH B-400, Pittsburgh, PA, 15213, USA.
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11
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Zhou LB, Zhang HC, Dong ZG, Wang CC. Chondrosarcoma of the toe: A case report and literature review. World J Clin Cases 2022; 10:9132-9141. [PMID: 36157642 PMCID: PMC9477028 DOI: 10.12998/wjcc.v10.i25.9132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2022] [Revised: 06/25/2022] [Accepted: 07/22/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Chondrosarcoma of the foot is a rare malignant bone tumour, and it is even rarer when it originates in a toe bone. Surgical excision is the only effective treatment. The osteolytic destruction of the tumour severely affects limb function and carries the risk of distant metastasis. Most such tumours are removed surgically to minimize local recurrence and distant metastases, maximize limb function, and prolong the patient's tumour-free survival time. The main objective of this article is to present the case of a chondrosarcoma that invaded the first phalanx of the left foot and formed a large phalangeal mass with osteolytic destruction of the distal bone.
CASE SUMMARY A 74-year-old man suffered from swelling of his left toe for six months, with pain and swelling for two months. Computed tomography and magnetic resonance imaging showed that the tumour on the first phalanx of the left foot was approximately 54.9 mm × 44.6 mm, surrounded by a significant soft tissue signal mass, with osteolytic destruction of the distal phalanx and a speckled bone-like high-density shadow within it.
CONCLUSION Chondrosarcoma occurring in a toe bone is extremely rare. In this case, extensive surgical resection of the large low-grade chondrosarcoma, which showed osteolytic destruction and invaded the distal metatarsal bone, was safe and effective.
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Affiliation(s)
- Li-Bo Zhou
- Graduate School, Qinghai University, Xining 810016, Qinghai Province, China
| | - He-Cheng Zhang
- Graduate School, Qinghai University, Xining 810016, Qinghai Province, China
| | - Zai-Gang Dong
- Graduate School, Qinghai University, Xining 810016, Qinghai Province, China
| | - Chao-Chao Wang
- Department of Bone and Joint Surgery, the Affiliated Hospital of Qinghai University, Xining 810012, Qinghai Province, China
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12
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Dehner CA, Maloney N, Amini B, Jennings JW, McDonald DJ, Wang WL, Chrisinger JSA. Dedifferentiated chondrosarcoma with minimal or small dedifferentiated component. Mod Pathol 2022; 35:922-928. [PMID: 35115636 DOI: 10.1038/s41379-022-01008-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2021] [Revised: 01/05/2022] [Accepted: 01/06/2022] [Indexed: 11/09/2022]
Abstract
Dedifferentiated chondrosarcoma (DDCS) is an aggressive bone sarcoma characterized by low-intermediate grade cartilage component with abrupt transition to a high-grade non-chondrosarcomatous component. Generally, the dedifferentiated (DD) component is large. However, rare cases have minimal (<1 cm) or small (1-2 cm) areas of DD. We describe the clinicopathologic features of such tumors and evaluate the prognostic significance of this finding compared to cases with large DD (>2 cm). Available slides were re-reviewed for assessment of histologic features. The medical record was reviewed for imaging studies and clinical characteristics. Thirty-five cases were included. Six patients had minimal DD, four had small DD and 25 had large DD. None of the minimal DD showed definitive imaging evidence of DD. Two minimal DD (33%) locally recurred and 2 (33%) developed distant metastases. None of the small DD cases showed definitive imaging evidence of DD. None of the small DD locally recurred and at least 1 (25%) developed distant metastases. There was no significant difference in age, gender, pelvic site, tumor size >8 cm, tumor necrosis or undifferentiated pleomorphic sarcoma-like morphology between minimal or small DD compared to large DD, though osteosarcomatous differentiation was significantly more common in large DD. There was no significant difference in overall survival between minimal or small DD compared to large DD (p = 0.81 and p = 0.17, respectively), or in progression-free survival (p = 0.47 and 0.29, respectively), or metastasis-free survival (p = 0.06 and 0.62, respectively). DDCS with minimal or small DD show similar demographic distribution, anatomic localization and histologic features to large DD. DD in these cases is unlikely to be detected on imaging. Furthermore, at least a subset of these tumors is extremely aggressive despite the limited extent of DD. This highlights the need for thorough gross and histologic examination and sampling.
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Affiliation(s)
- Carina A Dehner
- Department of Pathology and Immunology, Washington University in St. Louis School of Medicine, St. Louis, MO, USA
| | - Nolan Maloney
- Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Behrang Amini
- Department of Diagnostic Imaging, University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Jack W Jennings
- Musculoskeletal Section, Department of Radiology, Mallinckrodt Institute of Radiology, Washington University in St. Louis School of Medicine, St. Louis, MO, USA
| | - Douglas J McDonald
- Department of Orthopedic Surgery, Washington University in St. Louis School of Medicine, St. Louis, MO, USA
| | - Wei-Lien Wang
- Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - John S A Chrisinger
- Department of Pathology and Immunology, Washington University in St. Louis School of Medicine, St. Louis, MO, USA.
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Russo S, Scotto di Carlo F, Gianfrancesco F. The Osteoclast Traces the Route to Bone Tumors and Metastases. Front Cell Dev Biol 2022; 10:886305. [PMID: 35646939 PMCID: PMC9139841 DOI: 10.3389/fcell.2022.886305] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Accepted: 03/24/2022] [Indexed: 12/31/2022] Open
Abstract
Osteoclasts are highly specialized cells of the bone, with a unique apparatus responsible for resorption in the process of bone remodeling. They are derived from differentiation and fusion of hematopoietic precursors, committed to form mature osteoclasts in response to finely regulated stimuli produced by bone marrow-derived cells belonging to the stromal lineage. Despite a highly specific function confined to bone degradation, emerging evidence supports their relevant implication in bone tumors and metastases. In this review, we summarize the physiological role of osteoclasts and then focus our attention on their involvement in skeletal tumors, both primary and metastatic. We highlight how osteoclast-mediated bone erosion confers increased aggressiveness to primary tumors, even those with benign features. We also outline how breast and pancreas cancer cells promote osteoclastogenesis to fuel their metastatic process to the bone. Furthermore, we emphasize the role of osteoclasts in reactivating dormant cancer cells within the bone marrow niches for manifestation of overt metastases, even decades after homing of latent disseminated cells. Finally, we point out the importance of counteracting tumor progression and dissemination through pharmacological treatments based on a better understanding of molecular mechanisms underlying osteoclast lytic activity and their recruitment from cancer cells.
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Affiliation(s)
| | | | - Fernando Gianfrancesco
- Institute of Genetics and Biophysics “Adriano Buzzati-Traverso”, National Research Council of Italy, Naples, Italy
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14
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Sharif B, Lindsay D, Saifuddin A. Update on the imaging features of the enchondromatosis syndromes. Skeletal Radiol 2022; 51:747-762. [PMID: 34302201 DOI: 10.1007/s00256-021-03870-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2021] [Revised: 07/12/2021] [Accepted: 07/14/2021] [Indexed: 02/02/2023]
Abstract
Ollier disease and Maffucci syndrome are the commonest enchondromatosis subtypes, arising from non-hereditary mutations in the IDH1 and IDH2 genes, presenting in childhood and being characterised by multiple enchondromas. Maffucci syndrome also includes multiple soft tissue haemangiomas. Aside from developing bony masses, osseous deformity and pathological fracture, ~ 40% of these patients develop secondary central chondrosarcoma, and there is increased risk of non-skeletal malignancies such as gliomas and mesenchymal ovarian tumours. In this review, we outline the molecular genetics, pathology and multimodality imaging features of solitary enchondroma, Ollier disease and Maffucci syndrome, along with their associated skeletal complications, in particular secondary chondrosarcoma. Given the lifelong risk of malignancy, imaging follow-up will also be explored. Metachondromatosis, a rare enchondromatosis subtype characterised by enchondromas and exostoses, will also be briefly outlined.
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Affiliation(s)
- Ban Sharif
- Imaging Department, Northwick Park Hospital, Harrow, UK.
| | - Daniel Lindsay
- Pathology Department, Royal National Orthopaedic Hospital, Stanmore, UK
| | - Asif Saifuddin
- Imaging Department, Royal National Orthopaedic Hospital, Stanmore, UK
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15
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Pires V, Cavaca RP, Oliveira RC, Marques C. Sedimentation sign: a classical finding on tumorous calcinosis. BMJ Case Rep 2022; 15:e247613. [PMID: 35321912 PMCID: PMC8943780 DOI: 10.1136/bcr-2021-247613] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/16/2022] [Indexed: 11/04/2022] Open
Abstract
The authors describe the case of a man in his 60s who presented with progressive pain and swelling of the right hip. Imaging features showed a densely calcified lesion associated with 'sedimentation sign'. Laboratory tests revealed slight hyperphosphataemia. Surgical excision of the lesion was performed. Histological examination revealed chalky material surrounded by fibrosis and giant multinucleated cells, compatible with tumorous calcinosis. Patient made a full recovery. We report a rare case of tumorous calcinosis and compare this condition with its common mimics.
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Affiliation(s)
- Verena Pires
- Radiology, Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal
| | - Rita Pereira Cavaca
- Orthopaedics and Traumatology, Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal
| | | | - Cristina Marques
- Radiology, Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal
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16
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Bone Tumors. Radiol Clin North Am 2022; 60:221-238. [DOI: 10.1016/j.rcl.2021.11.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
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17
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Kattepur AK, Jones RL, Gulia A. Dedifferentiated chondrosarcoma: current standards of care. Future Oncol 2021; 17:4983-4991. [PMID: 34734747 DOI: 10.2217/fon-2021-0830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
Dedifferentiated chondrosarcomas are aggressive variants of chondrosarcoma, associated with poor outcomes. Tumor biphasism is the norm. The majority of these tumors are symptomatic at presentation. Radiologically, large soft tissue masses with bony destruction predominate. Treatment protocols of these tumors are not well defined. Surgical resection forms the standard of care for localized disease. (Neo)adjuvant therapies remain controversial as the results from multiple (mainly retrospective) studies remain conflicting. Age at presentation, stage and ability to obtain negative resection margins are important prognostic factors. The overall prognosis is dismal. Newer and novel therapies targeting the complex genetic makeup of these tumors have renewed interest in the adjuvant setting that could hold promise in the near future.
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Affiliation(s)
- Abhay K Kattepur
- Department of Surgical Oncology, Sri Devaraj Urs Academy of Higher Education & Research, Tamaka, Kolar 563101, Karnataka, India
| | - Robin L Jones
- Consultant Medical Oncologist, Royal Marsden Hospital NHS Trust, 203 Fulham Rd, London SW3 6JJ, UK
| | - Aashish Gulia
- Department of Surgical Oncology, Tata Memorial Hospital & Homi Bhabha National Institute (HBNI), Parel, Mumbai 400012, India
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18
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Alqubaisi A, Oliveira I, Singla N, Chavda A, Khoo M, Saifuddin A. The incidence and diagnostic relevance of pathological fracture in conventional central chondrosarcoma. Skeletal Radiol 2021; 50:1131-1140. [PMID: 33123743 DOI: 10.1007/s00256-020-03651-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Revised: 09/29/2020] [Accepted: 10/13/2020] [Indexed: 02/02/2023]
Abstract
OBJECTIVE To determine the incidence and diagnostic relevance of pathological fracture in patients with conventional central chondrosarcoma (CC-CS). MATERIALS AND METHODS Retrospective review of patients with CC-CS diagnosed between January 2007 and December 2019. Data collected included age, sex, skeletal location and the presence of pathological fracture at presentation. Histological tumour grade based on surgical resection or needle biopsy was classified as atypical cartilaginous tumour (ACT)/grade 1 CS (ACT/Gd1 CS), high-grade CS (HG-CS) and dedifferentiated CS (DD-CS). The presence of pathological fracture was correlated with age, skeletal location and tumour grade. RESULTS Three hundred seventeen patients were included (177 males and 140 females with mean age 55.8 years, range 9-91 years). Mean age of patients without pathological fracture was 54.4 years and those with pathological fracture 62.9 years (p = 0.002). The major long bones were involved in 171 cases, the flat bones in 112 cases, the mobile spine in 7 cases and the small bones of the hands and feet in 27 cases. There were 81 ACT/Gd 1 CS, 168 HGCS and 68 DD-CS. Pathological fracture was evident at presentation in 51 (16.1%) cases, the commonest bones involved being the femur (n = 21; 41.2%), humerus (n = 10; 19.6%) and acetabulum (n = 7; 13.7%). Pathological facture occurred in 7 cases of ACT/Gd 1 CS (13.7%), 23 cases of HGCS (45.1%) and 21 cases of DD-CS (41.2%) (p = 0.001). Following multivariate analysis, both older age and histological grade were independently significant factors. CONCLUSIONS Pathological fracture was seen in 16.1% of patients with CC-CS. Pathological fractures in the femur, humerus and acetabulum very likely indicate higher tumour grade.
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Affiliation(s)
- Abdulaziz Alqubaisi
- Department of Radiology, Department of Medical Imaging, King Abdulaziz Medical City, Riyadh, Saudi Arabia
| | - Ines Oliveira
- Department of Radiology, London North West Healthcare NHS Trust, London, UK
| | - Nehal Singla
- Department of Radiology, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
| | - Anesh Chavda
- Department of Radiology, West Middlesex University Hospital and Chelsea and Westminster Hospital, London, UK
| | - Michael Khoo
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.
| | - Asif Saifuddin
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK
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19
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Zając AE, Kopeć S, Szostakowski B, Spałek MJ, Fiedorowicz M, Bylina E, Filipowicz P, Szumera-Ciećkiewicz A, Tysarowski A, Czarnecka AM, Rutkowski P. Chondrosarcoma-from Molecular Pathology to Novel Therapies. Cancers (Basel) 2021; 13:2390. [PMID: 34069269 PMCID: PMC8155983 DOI: 10.3390/cancers13102390] [Citation(s) in RCA: 46] [Impact Index Per Article: 11.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2021] [Revised: 05/01/2021] [Accepted: 05/04/2021] [Indexed: 12/16/2022] Open
Abstract
Chondrosarcoma (CHS) is the second most common primary malignant bone sarcoma. Overall survival and prognosis of this tumor are various and often extreme, depending on histological grade and tumor subtype. CHS treatment is difficult, and surgery remains still the gold standard due to the resistance of this tumor to other therapeutic options. Considering the role of differentiation of CHS subtypes and the need to develop new treatment strategies, in this review, we introduced a multidisciplinary characterization of CHS from its pathology to therapies. We described the morphology of each subtype with the role of immunohistochemical markers in diagnostics of CHS. We also summarized the most frequently mutated genes and genome regions with altered pathways involved in the pathology of this tumor. Subsequently, we discussed imaging methods and the role of currently used therapies, including surgery and the limitations of chemo and radiotherapy. Finally, in this review, we presented novel targeted therapies, including those at ongoing clinical trials, which can be a potential future target in designing new therapeutics for patients with CHS.
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Affiliation(s)
- Agnieszka E. Zając
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
| | - Sylwia Kopeć
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
| | - Bartłomiej Szostakowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
| | - Mateusz J. Spałek
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
| | - Michał Fiedorowicz
- Small Animal Magnetic Resonance Imaging Laboratory, Mossakowski Medical Research Institute, Polish Academy of Sciences, 02-106 Warsaw, Poland;
| | - Elżbieta Bylina
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
- Department of Clinical Trials, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland
| | - Paulina Filipowicz
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
- Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Anna Szumera-Ciećkiewicz
- Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland;
- Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, 02-776 Warsaw, Poland
| | - Andrzej Tysarowski
- Department of Pathology and Laboratory Medicine, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland;
- Department of Molecular and Translational Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland
| | - Anna M. Czarnecka
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
| | - Piotr Rutkowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland; (A.E.Z.); (S.K.); (B.S.); (M.J.S.); (E.B.); (P.F.); (P.R.)
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20
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Gaeta R, Aringhieri G, Zampa V, D'Arienzo A, Andreani L, Capanna R, Zavaglia K, Franchi A. Acral Dedifferentiated Chondrosarcoma: Report of a Case Arising in the Proximal Phalanx of the Fourth Finger. Int J Surg Pathol 2021; 29:892-898. [PMID: 33847539 DOI: 10.1177/10668969211010221] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Dedifferentiated chondrosarcoma is a well-recognized entity, but its occurrence in the distal extremities is exceedingly rare. We present the case of a 49-year-old woman who experienced local recurrence of an "enchondroma" of the proximal phalanx of the fourth finger of the left hand, which had been initially treated with intralesional curettage at another hospital 4 years before, and 1 year before for a local recurrence. The imaging findings indicated an aggressive behavior, and an incisional biopsy showed a highly cellular proliferation of spindle and pleomorphic elements without evidence of matrix production intermixed with few fragments of a well-differentiated cartilaginous neoplasm with bland cellular atypia, focal nuclear hyperchromatism, and binucleation. An isocitrate dehydrogenase 2 R172S mutation was detected. The final diagnosis was dedifferentiated chondrosarcoma. Despite amputation of the fourth finger, the patient developed lung metastases and further local relapse. Recurrent cartilaginous tumors of the extremities should not be underestimated and should be followed in view of the possible acquisition of aggressive clinical behavior.
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Affiliation(s)
- Raffaele Gaeta
- 9310Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy
| | - Giacomo Aringhieri
- 9310Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy
| | - Virna Zampa
- Department of Diagnostics and Imaging, Pisa University Hospital, Pisa, Italy
| | - Antonio D'Arienzo
- 2nd Orthopedic and Traumatology Clinic, Pisa University Hospital, Pisa, Italy
| | - Lorenzo Andreani
- 2nd Orthopedic and Traumatology Clinic, Pisa University Hospital, Pisa, Italy
| | - Rodolfo Capanna
- 9310Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy
| | - Katia Zavaglia
- Division of Molecular Genetics, Department of Laboratory Medicine, Pisa University Hospital, Pisa, Italy
| | - Alessandro Franchi
- 9310Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy
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21
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Cao L, Wu Y, Han SM, Sun T, Yu BH, Gao F, Wu WJ, Gao BL. Dedifferentiated chondrsarcoma: a clinicopathologic analysis of 25 cases. BMC Musculoskelet Disord 2021; 22:189. [PMID: 33588810 PMCID: PMC7885579 DOI: 10.1186/s12891-021-04053-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2020] [Accepted: 02/04/2021] [Indexed: 11/10/2022] Open
Abstract
Background To investigate the clinical, imaging and pathological features of dedifferentiated chondrosarcoma for better diagnosis. Methods Patients who had been confirmed to have dedifferentiated chondrosarcoma were enrolled in this study and analyzed in the clinical, imaging and pathological data. Results Twenty-five patients had pathologically confirmed dedifferentiated chondrosarcoma including 15 males and 10 females with an age range of 24–74 (median 58, interquartile range 49–65). Ten patients had the tumor at the femur, four at the ilium, two at the humerus, two at the tibia, two at cotyle, and one at each of the following locations: scapula, sacrum, rib, pubic branch, and calcaneus. Twenty-one patients had local pain and a soft tissue mass while the other four patients had only local pain without a soft tissue mass. Four patients had pathological fractures. Imaging showed extensive bone destruction with calcification inside the lesion and possible pathological fractures. On gross observation of the specimen, the chondrosarcoma components were usually located inside the bone, and the dedifferentiated sarcoma components were mainly located outside the bone. Microscopy showed the dedifferentiated tumor had two components: well-differentiated chondrosarcoma and poorly differentiated non-chondral sarcoma including malignant fibrous histiocytoma in eleven cases, osteosarcoma in ten cases, fibrosarcoma in two, liomyosarcoma in one, and lipoblastoma in the remaining one.. Followed up from 3 moths to 60 months (mean 15.6), eight patients died with a survival time of 10–23 months (mean 16), and the other 17 patients survived with the survival duration from three to 60 months (15). Conclusion Dedifferentiated chondrosarcoma is a fatal disease with multiple components, and most of the cases have dual morphological and imaging features of chondrosarcoma and non-chondrosarcoma. The imaging presentations are primarily of common central chondrosarcoma, combined with cortical destruction, soft tissue mass, and pathological fractures.
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Affiliation(s)
- Lei Cao
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
| | - Yuan Wu
- Hebei Provincial Gucheng County Hospital, Gucheng, 253800, Hebei Province, China
| | - Shu-Man Han
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
| | - Tao Sun
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
| | - Bao-Hai Yu
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
| | - Feng Gao
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
| | - Wen-Juan Wu
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China.
| | - Bu-Lang Gao
- Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China
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22
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Sharif B, Lindsay D, Saifuddin A. The role of imaging in differentiating low-grade and high-grade central chondral tumours. Eur J Radiol 2021; 137:109579. [PMID: 33578088 DOI: 10.1016/j.ejrad.2021.109579] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2020] [Revised: 01/23/2021] [Accepted: 01/31/2021] [Indexed: 12/16/2022]
Abstract
Chondrosarcoma is a malignant cartilage matrix-producing tumour. Those arising de novo are called primary chondrosarcomas and are the second commonest primary malignant bone tumours. Numerous types of primary chondrosarcoma exist, namely conventional central (intramedullary), periosteal (juxta-cortical), clear cell, mesenchymal, and dedifferentiated. The biologic aggressiveness, prognosis and thus management of chondrosarcoma are dependent on the histological sub-type and grade. Accurate pre-operative diagnosis is therefore essential in determining management and outcome which requires a multidisciplinary approach taking into account clinical features, imaging findings and histopathology. In this review, we present the pertinent multimodality imaging features which aid in the differentiation of low-grade and high-grade conventional central chondrosarcoma.
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Affiliation(s)
- Ban Sharif
- Imaging Department, Northwick Park Hospital, United Kingdom.
| | - Daniel Lindsay
- Pathology Department, Royal National Orthopaedic Hospital, United Kingdom
| | - Asif Saifuddin
- Imaging Department, Royal National Orthopaedic Hospital, United Kingdom
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23
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Saifuddin A, Oliveira I, Singla N, Chavda A, Khoo M, O'Donnell P. The importance of MRI review following the diagnosis of atypical cartilaginous tumour using image-guided needle biopsy. Skeletal Radiol 2021; 50:407-415. [PMID: 32794057 DOI: 10.1007/s00256-020-03578-7] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/08/2020] [Revised: 08/08/2020] [Accepted: 08/09/2020] [Indexed: 02/02/2023]
Abstract
OBJECTIVE To evaluate the role of pre-biopsy MRI for management planning in patients with atypical cartilaginous tumours (ACT) diagnosed on image-guided core needle biopsy (IGCNB). MATERIALS AND METHODS Retrospective review of patients diagnosed with ACT of the appendicular skeleton based on IGCNB who subsequently underwent surgical curettage or resection. Data collected included age, sex, skeletal location and surgical histology classified as ACT, high-grade chondrosarcoma (HG-CS) and dedifferentiated chondrosarcoma (DD-CS). Pre-biopsy MRI studies were reviewed independently by 2 radiologists blinded to surgical histology results and graded as ACT, HG-CS and DD-CS based on MRI features. RESULTS The study included 24 males and 28 females (mean age 42.1 years; range 9-76 years). One patient had 2 lesions treated, making a total of 53 lesions. Tumours were located in the femur in 21 cases, humerus in 17, tibia in 9, radius in 4 and ulna and fibula in 1 each. Surgical histology was ACT in 41 cases, HG-CS in 10 and DD-CS in 2, indicating mismatch between IGCNB and surgical histology in 12/53 cases (22.6%). Predicted MRI grade for the 2 readers was ACT in 39 cases each, HG-CS in 13 and 14 cases and DD-CS in 1 and 0 cases. Sensitivity, specificity and accuracy of MRI for predicting HG-CS/DD-CS were 91%, 93% and 92%, respectively. Inter-observer correlation was very good (kappa = 0.94). DISCUSSION Review of MRI findings in patients with ACT diagnosed on IGCNB is vital for identifying patients with a HG-CS/DD-CS and is recommended when planning surgical management or considering repeat IGCNB.
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Affiliation(s)
- Asif Saifuddin
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK
| | - Ines Oliveira
- Department of Radiology, London North West Healthcare NHS Trust, London, UK
| | - Nehal Singla
- Department of Radiology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
| | - Anesh Chavda
- Department of Radiology, West Middlesex University Hospital and Chelsea and Westminster Hospital, London, UK
| | - Michael Khoo
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.
| | - Paul O'Donnell
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.,Institute of Orthopaedics and Musculoskeletal Science, University College London, London, UK
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Mustaki L, Goetti P, Gallusser N, Morattel B, Rüdiger HA, Cherix S. Unrecognized Chondrosarcoma as a Cause of Total Hip Arthroplasty Failure. Arthroplast Today 2021; 7:84-90. [PMID: 33521202 PMCID: PMC7818612 DOI: 10.1016/j.artd.2020.12.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2020] [Revised: 11/20/2020] [Accepted: 12/07/2020] [Indexed: 11/30/2022] Open
Abstract
Background Total hip arthroplasty (THA) is one of the most successful procedures in orthopedic surgery. The most frequent THA indications are osteoarthritis and avascular necrosis, whereas symptomatic aseptic loosening is the most common indication to revision surgery. Chondrosarcoma (CS) is the most frequent bone sarcoma in adults, and proximal femur is the most prevalent location. Wide resection is the treatment of choice.We report 3 cases of unrecognized high-grade CS in the setting of primary or revision THA and reviewed the literature on this rare clinical presentation. Methods A systematic literature review on CS in the setting of THA, published between 1980 and 2020, was performed on PubMed, Embase, Medline, Ovid SP, and Web of Science, using the guidelines set in the Preferred Reporting Items for Systematic Reviews and Mata-analyses (PRISMA). Results Case series: Three patients were referred to our sarcoma center after failure of THA due to unrecognized high-grade CS. All 3 had rapid fatal outcome. Literature review: Fifty-nine articles were identified, of which 8 were included in the study. They confirmed that primary or revision THA failure due to unrecognized CS is extremely rare, with only few cases reported in the literature. Conclusions Before proceeding to primary or revision arthroplasty, diagnosis must be ascertained. Atypical presentation of a common pathology, such as osteoarthritis, avascular necrosis, or aseptic loosening of an endoprosthesis, should raise suspicion for another cause to symptoms, and additional workup be performed. As our cases demonstrated, unrecognized or inadequately managed bone sarcoma may lead to poor or even fatal outcome.
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Affiliation(s)
- Laurent Mustaki
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Patrick Goetti
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Nicolas Gallusser
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Boris Morattel
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Hannes A Rüdiger
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.,Department of Orthopeadics, Schulthess Clinic, Zürich, Switzerland
| | - Stéphane Cherix
- Department of Orthopaedics and Traumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
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The value of chest and skeletal staging studies in conventional chondrosarcoma. Skeletal Radiol 2021; 50:125-135. [PMID: 32666323 DOI: 10.1007/s00256-020-03539-0] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Revised: 07/02/2020] [Accepted: 07/03/2020] [Indexed: 02/02/2023]
Abstract
OBJECTIVE To determine the value of chest and skeletal staging in patients presenting with conventional chondrosarcoma (CS). MATERIALS AND METHODS Retrospective review of patients with CS diagnosed between January 2007 and December 2019. Data collected included age, sex, skeletal location and results of chest CT and whole-body bone scintigraphy (WB-BSc) obtained at initial diagnosis. The histological tumour grade based on surgical resection or needle biopsy was classified as low-grade (LGCT), high-grade (HGCT) and dedifferentiated (DD-CS). Findings of chest CT and WB-BSc were correlated with tumour grade. RESULTS Four hundred twenty patients were included (234 males and 186 females with mean age 54.5 years, range 9-91 years). The major long bones were involved in 205 cases, the flat bones in 166 cases, the mobile spine in 14 cases and the small bones of the hands and feet in 35 cases. Three hundred fifty tumours were central in location, 39 peripheral and 31 on the surface of the bone. There were 151 LGCTs, 196 HGCTs and 73 DD-CSs. Of patients with LGCT, 41.7% underwent chest CT and 25.2% WB-BSc. Of patients with HGCT, 95.4% underwent chest CT and 76.5% WB-BSc. Of patients with DD-CS, 98.6% underwent chest CT and 86.3% WB-BSc. Metastases were diagnosed in 2 (3.3%) chest CT studies and 0 WB-BSc in LGCT, in 8 (4.3%) chest CT studies and 0 WB-BSc in HGCT and in 21 (30%) chest CT studies and 6 (21%) WB-BSc in DD-CS. DISCUSSION Staging chest CT and WB-BSc is of little or no value except in DD-CS.
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Gomez CD, Anderson MS, Epperly SC, Zuckerman LM. Successful treatment of a dedifferentiated chondrosarcoma of the proximal humerus with a hemicortical articular surface sparing allograft: A case report. Int J Surg Case Rep 2020; 72:590-595. [PMID: 32698295 PMCID: PMC7330425 DOI: 10.1016/j.ijscr.2020.06.092] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2020] [Accepted: 06/22/2020] [Indexed: 11/25/2022] Open
Abstract
INTRODUCTION Dedifferentiated chondrosarcomas are rare and highly malignant tumors that require wide surgical resection. Survival is extremely poor without adequate surgical margins. We present a case of articular sparing surgery of the shoulder for dedifferentiated chondrosarcoma with excellent functional outcomes and no evidence of disease after fifty-six months. PRESENTATION OF CASE A 29-year-old male was found to have a non-metastatic right proximal humerus dedifferentiated chondrosarcoma. He underwent resection with clear one centimeter margins sparing the medial cortex and the articular surface. Reconstruction of the bone was accomplished using a hemicortical allograft. A dermal allograft was used to help reconstruct the rotator cuff to the allograft bone. At fifty-six months after surgery he has excellent functional range of motion. His current MSTS, Quick Dash, and Constant Shoulder scores are 29, 2.3, and 80, respectively. He has remained free of disease, is back to work without restrictions and is active in outdoor activities. DISCUSSION Dedifferentiated chondrosarcoma has high recurrence and poor survival rates. Adequate surgical resection is vital for its treatment. Previously described reconstructive techniques have consisted of articular replacement with a prosthesis, allograft, or allograft-prosthetic composites. To our knowledge, this is the first report of an articular sparing reconstruction for dedifferentiated chondrosarcoma with fifty-six month survival and functional outcomes. When possible, sparing the articular surface can provide good functional outcomes that improve over time. CONCLUSION If adequate surgical margins can be obtained, an articular surface sparing reconstruction of the shoulder can provide effective functional outcomes and an alternative to joint replacement.
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Affiliation(s)
- Charles D Gomez
- Department of Orthopedic Surgery, Loma Linda University, 11406 Loma Linda Dr, Loma Linda, CA 92354, USA.
| | - Mark S Anderson
- Department of Orthopedic Surgery, Loma Linda University, 11406 Loma Linda Dr, Loma Linda, CA 92354, USA.
| | - Scott C Epperly
- Department of Orthopedic Surgery, Loma Linda University, 11406 Loma Linda Dr, Loma Linda, CA 92354, USA.
| | - Lee M Zuckerman
- Department of Orthopedic Surgery, Loma Linda University, 11406 Loma Linda Dr, Loma Linda, CA 92354, USA.
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Oliveira I, Chavda A, Rajakulasingam R, Saifuddin A. Chondral tumours: discrepancy rate between needle biopsy and surgical histology. Skeletal Radiol 2020; 49:1115-1125. [PMID: 32147756 DOI: 10.1007/s00256-020-03406-y] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2019] [Revised: 02/26/2020] [Accepted: 02/27/2020] [Indexed: 02/02/2023]
Abstract
OBJECTIVE To determine the reliability of image-guided core needle biopsy (IGCNB) for the diagnosis and grading of chondral tumours of bone compared with surgical histology. MATERIALS AND METHODS Retrospective review of patients with a chondral tumour of bone who underwent IGCNB and surgical resection between January 2007 and December 2017. Data collected included age, sex, skeletal location, technique used for IGCNB, IGCNB result including histological grade and comparison with surgical histology. RESULTS A total of 237 patients were included (135 males and 102 females with mean age 53.7 years, range 9-89 years). A total of 174 IGCNBs were CT-guided, 57 ultrasound-guided and 6 fluoroscopic-guided. Two hundred thirty-six of 237 (99.6%) IGCNBs were diagnostic for a chondral tumour, although grade could not be determined in 13 (5.5%) due to necrosis. A positive correlation for tumour grade between IGCNB and surgical histology was achieved in 181 cases (76.4%). In 36 patients (15.2%), IGCNB under-graded the tumour, while in 6 (2.5%), IGCNB over-graded the tumour. Discrepancy between IGCNB and surgical histology was significantly greater for surface/peripheral lesions (p = 0.02) and lesions arising from the flat bones or spine (p = 0.002). DISCUSSION IGCNB can achieve a diagnosis of a chondral tumour in a high proportion of cases when compared with final diagnosis from surgical resection specimens. However, correlation of tumour grade between IGCNB and resection histology is less reliable with discordance seen in almost one-quarter of cases, most commonly at non-appendicular sites. Therefore, IGCNB results should not be considered in isolation of imaging and clinical features when planning surgical management.
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Jo O, Schlicht S, Slavin J, Di Bella C, Pang G, Powell G, Spelman T, Choong PF. The role of Thallium-201 scintigraphy and Tc-99m pentavalent dimercaptosuccinic acid in diagnosis and grading of chondrosarcoma. Eur J Radiol 2020; 125:108846. [PMID: 32062570 DOI: 10.1016/j.ejrad.2020.108846] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2019] [Revised: 12/02/2019] [Accepted: 01/16/2020] [Indexed: 12/26/2022]
Abstract
PURPOSE Distinguishing between enchondromas and low-grade (grade 1) chondrosarcomas can be challenging. The aim of this study was to investigate the role of Thallium-201 scintigraphy and Technetium-99 m pentavalent dimercaptosuccinic acid (Tc-99 m DMSA (V)) in the diagnosis and grading of chondrosarcomas. METHODS 232 consecutive patients with pathologically proven cartilaginous tumours between the years 2000 and 2018 were evaluated. We included 197 patients (101 males and 96 females; median age 50 years; range 15-86 years) who underwent Thallium-201(n = 193) and/or Tc-99 m DMSA (V) scanning (n = 67). Increased uptake was defined as uptake greater than background. The reference standard was the histopathological assessment based on a grading system (grade 1-3). Data was analysed using multivariate modelling. RESULTS There were 46 patients with enchondromas and 151 with chondrosarcomas. Of those, 64 (enchondroma n = 21, chondrosarcoma n = 43) underwent both Thallium-201 and Tc-99 m DMSA (V). Thallium-201 uptake had 7.92 times greater odds of grade 1 chondrosarcomas than enchondromas. Thallium-201 uptake was significantly associated with the odds of a higher grade chondrosarcoma (grade 2-3). DMSA (V) positivity was associated with 4.75 times the odds of a chondrosarcoma diagnosis over enchondroma (p = 0.024). DMSA (V) uptake revealed no association with chondrosarcoma grading. CONCLUSION Low-grade chondrosarcomas continue to pose a diagnostic dilemma. Thallium-201 scans may identify malignancy in benign appearing tumours as well as differentiate between low-grade and high-grade chondrosarcomas in said malignancies. DMSA (V) may be useful in distinguishing between benign and malignant entities as a whole.
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Affiliation(s)
- Olivia Jo
- Melbourne Medical School, University of Melbourne, Victoria, Australia; Department of Orthopaedics, St. Vincent's Hospital, Melbourne, Victoria, Australia.
| | - Stephen Schlicht
- Department of Medical Imaging, St. Vincent's Hospital, Melbourne, Victoria, Australia
| | - John Slavin
- Department of Anatomical Pathology, St. Vincent's Hospital, Melbourne, Victoria, Australia
| | - Claudia Di Bella
- Department of Orthopaedics, St. Vincent's Hospital, Melbourne, Victoria, Australia; Department of Surgery, St. Vincent's Hospital, Melbourne, Victoria, Australia
| | - Grant Pang
- Department of Orthopaedics, St. Vincent's Hospital, Melbourne, Victoria, Australia
| | - Gerard Powell
- Department of Orthopaedics, St. Vincent's Hospital, Melbourne, Victoria, Australia
| | - Tim Spelman
- Department of Surgery, University of Melbourne, Victoria, Australia
| | - Peter Fm Choong
- Department of Orthopaedics, St. Vincent's Hospital, Melbourne, Victoria, Australia; Department of Surgery, St. Vincent's Hospital, Melbourne, Victoria, Australia
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Grieser T. [Foot and ankle tumours : Part II: Malignant bone tumours and soft tissue tumours of the foot with differential diagnostic hints]. Radiologe 2019; 58:459-475. [PMID: 29492589 DOI: 10.1007/s00117-018-0361-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
CLINICAL ISSUE Both benign and malignant tumours are encountered in the foot and ankle. Due to their rarity, however, diagnosis is often uncertain. Usual criteria such as tumour size, invasiveness or pain fail to differentiate benign from malignant neoplasias. STANDARD RADIOLOGICAL METHODS Plain radiography and-due to the complex foot anatomy-CT are important in the diagnostic evaluation of primary bone tumours. In the case of soft tissue tumours, ultrasonography is used to identify ganglion cysts. Tumour evaluation and staging are then performed using MRI. Nuclear imaging comes into play when multifocal, metastatic or systemic spread of the disease is suspected or if a biopsy procedure is planned. METHODICAL INNOVATIONS For the role of dual-energy CT, please refer to part I. Whether dual-energy CT is suitable to assess bone marrow oedema patterns is still debated. SPECIAL FEATURES OF FOOT AND ANKLE TUMOURS Primary bone tumours generally present in the ankle or the hindfoot. Malignant bone tumours, e. g. chondrosarcoma, are primarily found in the latter. On the other hand, soft tissue tumours generally present in the mid- and forefoot regions. ACHIEVEMENTS While the radiologic diagnostic evaluation of pedal bone tumours is quite reliable, caution is urgently needed in the case of soft tissue tumours because there are many confusing differential diagnoses. PRACTICAL RECOMMENDATIONS Proper X‑ray examination of the foot (at least in two plains) or with special views according to bony pathology is needed. All soft tissue masses that are not cysts should be evaluated further. MRI remains the diagnostic modality of choice but never stands alone! Caution: Size and well-defined margins of pedal soft tissue tumours are not considered criteria regarding whether a tumour is malignant or not.
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Affiliation(s)
- T Grieser
- Klinik für Diagnostische und Interventionelle Radiologie und Neuroradiologie, Klinikum Augsburg, Stenglinstraße 2, 86156, Augsburg, Deutschland.
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Pandey S, Li L, Deng XY, Cui DM, Gao L. Outcome Following the Treatment of Ventriculitis Caused by Multi/Extensive Drug Resistance Gram Negative Bacilli; Acinetobacter baumannii and Klebsiella pneumonia. Front Neurol 2019; 9:1174. [PMID: 30728802 PMCID: PMC6352847 DOI: 10.3389/fneur.2018.01174] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2018] [Accepted: 12/18/2018] [Indexed: 12/02/2022] Open
Abstract
Introduction: CNS ventriculitis is a serious complication following an intracranial insult that demands immediate treatment with broad-spectrum antibiotics in a critical care setting. Infections due to multi/extensive drug resistance (MDR/XDR) microorganisms are very challenging, which may demand an additional approach to the ongoing practice; intravenous and intraventricular administration of antibiotics. Aim: To study the efficacy and safety of thorough ventricular irrigation followed by daily intraventricular antibiotic administration in patients with MDR/XDR ventriculitis. Materials and Methods: A retrospective analysis was done on 19 inpatients with ventriculitis caused by Acinetobacter baumannii (AB) or Klebsiella pneumonia (KP), at Shanghai Tenth People's Hospital from January 2016 to October 2017. We reviewed our experience; the role of thorough ventricular irrigation with Colistin mixed normal saline, followed by intraventricular Colistin therapy. Treatment outcomes were evaluated based on the clinical symptoms, Cerebro-Spinal Fluid (CSF) culture, laboratory findings and complications. Results: A total of 19 patients were included (15 males and 4 females), with a mean age in years of 51, which ranged from 18–67. Fourteen patients had Acinetobacter baumannii (AB) and 5 had Klebsiella pneumoniae (KP). The average CSF sterilization period following ventricular irrigation and intraventricular Colistin was 6 days. Sixteen patients (84%) were cured, and 3 patients (15%) died during the course of the treatment. Conclusion: In addition to Intraventricular Colistin, thorough ventricular irrigation could increase the cure rate up to 84% in patients suffering from MDR/XDR CNS ventriculitis.
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Affiliation(s)
- Sajan Pandey
- Neurosurgery Department, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China.,Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
| | - Lei Li
- Neurosurgery Department, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China.,Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
| | - Xian Yu Deng
- Neurosurgery Department, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China.,Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
| | - Da Ming Cui
- Neurosurgery Department, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
| | - Liang Gao
- Neurosurgery Department, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China.,Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
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Lex JR, Evans S, Stevenson JD, Parry M, Jeys LM, Grimer RJ. Dedifferentiated chondrosarcoma of the pelvis: clinical outcomes and current treatment. Clin Sarcoma Res 2018; 8:23. [PMID: 30559960 PMCID: PMC6293503 DOI: 10.1186/s13569-018-0110-1] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2018] [Accepted: 12/04/2018] [Indexed: 12/13/2022] Open
Abstract
Background Dedifferentiated chondrosarcomas (CS) are a high-grade variant of CS that confers a 5-year survival of around 10–24%. Dedifferentiated CS arising from the pelvis confers an even worse prognosis. Questions (1) What is the prognosis of patients with dedifferentiated CS of the pelvis? (2) Do wide margins or type of surgical intervention influence outcome? (3) Does the use of adjuvant therapy affect outcome? Methods Patients were retrospectively reviewed from a prospectively collated musculoskeletal oncology database from 1995 to 2016. Thirty-one cases of dedifferentiated CS arising from the pelvis were included. Wide margins were defined as greater than 4 mm. The mean age was 55.6 years (range 33 to 76 years) and there were 19 males (61.3%) and 12 females (38.7%). Results The disease presented at a locally or systemically advanced stage in 13 patients (41.9%). Eighteen patients (58.1%) underwent surgery with curative intent. Overall survival at 12 months was 15.4% for patients treated with palliative intent and 50% for those treated with surgery. In the surgical group, there were higher rates of disease-free survival in patients who underwent hindquarter amputation and those who received wide surgical margins (p = 0.047 and p = 0.019, respectively). Those who underwent hindquarter amputation were more likely to achieve wide margins (p = 0.05). Time to recurrent disease (local or systemic) was always less than 24 months. No hindquarter amputation for recurrent disease resulted in disease-free survival. No patient who received adjuvant therapy for palliative or recurrent disease had disease control. Conclusions Pelvic dedifferentiated CS often presents at an advanced local or systemic stage and confers a poor prognosis. Achieving wide surgical margins (> 4 mm) provided the highest rate of long-term disease-free survival. Failing to achieve wide margins results in rapid disease recurrence, conferring deleterious consequences.
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Affiliation(s)
| | - Scott Evans
- 1Royal Orthopaedic Hospital, Birmingham, B31 2AP UK
| | | | | | - Lee M Jeys
- 1Royal Orthopaedic Hospital, Birmingham, B31 2AP UK.,2Aston University, Birmingham, UK
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Mosaic-pattern dedifferentiation in liposarcoma and chondrosarcoma: imaging features of an uncommon form of dedifferentiation. Skeletal Radiol 2018; 47:877-882. [PMID: 29362843 DOI: 10.1007/s00256-017-2866-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/16/2017] [Revised: 12/26/2017] [Accepted: 12/27/2017] [Indexed: 02/02/2023]
Abstract
The term "dedifferentiation" was classically used in sarcoma pathology to refer to tumors in which a high-grade, undifferentiated sarcoma, or a high-grade sarcoma showing heterologous differentiation, arises from a pre-existing neoplasm of borderline or low-grade malignancy. The best recognized examples of this include dedifferentiated liposarcoma, arising from atypical lipomatous tumor/well-differentiated liposarcoma, and dedifferentiated chondrosarcoma, arising from grade 1 hyaline chondrosarcoma of bone. In the overwhelming majority of cases, this dedifferentiated, high-grade sarcoma presents as a macroscopically visible mass, adjacent to and clearly distinct from the pre-existing low-grade lesion. It is less well appreciated that dedifferentiation may also occur in a so-called "mosaic pattern,' in which the high-grade component is intimately admixed with elements of the precursor lesion, forming only microscopically apparent foci. This mosaic or co-mingling pattern of dedifferentiation is also reflected in the MR imaging appearance. In contrast to the classic pattern of dedifferentiation in which there are two distinct juxtaposed masses with different signal intensities and enhancement patterns, such changes are not seen in mosaic dedifferentiation. The imaging features of this pattern of dedifferentiation have not been described. In this report we describe the imaging features of two patients with mosaic pattern dedifferentiation, one with liposarcoma and one with chondrosarcoma. In both cases the precursor lesion was correctly diagnosed by pre-biopsy imaging, but the presence of high-grade sarcoma was not recognized.
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Redondo A, Bagué S, Bernabeu D, Ortiz-Cruz E, Valverde C, Alvarez R, Martinez-Trufero J, Lopez-Martin JA, Correa R, Cruz J, Lopez-Pousa A, Santos A, García Del Muro X, Martin-Broto J. Malignant bone tumors (other than Ewing's): clinical practice guidelines for diagnosis, treatment and follow-up by Spanish Group for Research on Sarcomas (GEIS). Cancer Chemother Pharmacol 2017; 80:1113-1131. [PMID: 29038849 PMCID: PMC5686259 DOI: 10.1007/s00280-017-3436-0] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2017] [Accepted: 09/15/2017] [Indexed: 12/17/2022]
Abstract
Primary malignant bone tumors are uncommon and heterogeneous malignancies. This document is a guideline developed by the Spanish Group for Research on Sarcoma with the participation of different specialists involved in the diagnosis and treatment of bone sarcomas. The aim is to provide practical recommendations with the intention of helping in the clinical decision-making process. The diagnosis and treatment of bone tumors requires a multidisciplinary approach, involving as a minimum pathologists, radiologists, surgeons, and radiation and medical oncologists. Early referral to a specialist center could improve patients' survival. The multidisciplinary management of osteosarcoma, chondrosarcoma, chordoma, giant cell tumor of bone and other rare bone tumors is reviewed in this guideline. Ewing's sarcoma will be the focus of a separate guideline because of its specific biological, clinical and therapeutic features. Each statement has been accompanied by the level of evidence and grade of recommendation on the basis of the available data. Surgical excision is the mainstay of treatment of a localized bone tumor, with various techniques available depending on the histologic type, grade and location of the tumor. Chemotherapy plays an important role in some chemosensitive subtypes (such as high-grade osteosarcoma). In other subtypes, historically considered chemoresistant (such as chordoma or giant cell tumor of bone), new targeted therapies have emerged recently, with a very significant efficacy in the case of denosumab. Radiation therapy is usually necessary in the treatment of chordoma and sometimes of other bone tumors.
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Affiliation(s)
- Andrés Redondo
- Hospital La Paz, Paseo Castellama, 261, 28046, Madrid, Spain.
| | - Silvia Bagué
- Hospital de la Santa Creu i Sant Pau, Carrer de Sant Quintí, 89, 08026, Barcelona, Spain
| | - Daniel Bernabeu
- Hospital La Paz, Paseo Castellama, 261, 28046, Madrid, Spain
| | | | - Claudia Valverde
- Hospital Vall d'Hebrón, Passeig de la Vall d'Hebrón 119-129, 08035, Barcelona, Spain
| | - Rosa Alvarez
- Hospital Gregorio Marañón, C/ Dr Esquerdo 46, 28007, Marid, Spain
| | | | | | - Raquel Correa
- Hospital Virgen de la Victoria, Campus de Teatinos s/nº, 29010, Málaga, Spain
| | - Josefina Cruz
- Hospital Universitario de Canarias, Carretera de Ofra s/n, 38320, San Cristóbal de la Laguna, Santa Cruz De Tenerife, Spain
| | - Antonio Lopez-Pousa
- Hospital de la Santa Creu i Sant Pau, C/. Mas Casanovas, 90, 08041, Barcelona, Spain
| | - Aurelio Santos
- Hospital Virgen del Rocío, Av Manuel Siurot s/n, 41013, Sevilla, Spain
| | - Xavier García Del Muro
- Institut Catalá d'Oncologia Hospitalet, Avinguda de la GranVia de l´Hospitalet 199-203, L´Hospitalet de Llobregat, Barcelona, Spain
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Chen L, Yu Z, Jiang R, Dong P, Shen B, Li Y. A case of dedifferentiated chondrosarcoma arising in the cricoid cartilage that mimicked an aneurysmal bone cyst. Postgrad Med 2017; 130:274-277. [PMID: 29160138 DOI: 10.1080/00325481.2018.1408382] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Dedifferentiated chondrosarcoma of the larynx is a rare and highly malignant tumor. We present the report of a 59-year-old man with dedifferentiated laryngeal chondrosarcoma, which was difficult to diagnose even under microscopic examination. The original diagnosis was an aneurysmal bone cyst, and the final diagnosis was established only after careful consideration of the imaging, surgical, and microscopic findings. In clinical practice, there are many similarities between dedifferentiated chondrosarcoma and aneurysmal bone cysts. Furthermore, it is difficult to identify dedifferentiated laryngeal chondrosarcoma with a giant-cell malignant mesenchymal component. This report describes our experience and discusses this phenomenon.
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Affiliation(s)
- Lixiao Chen
- a Department of Otolaryngology , Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , P.R. China
| | - Ziwei Yu
- a Department of Otolaryngology , Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , P.R. China
| | - Rui Jiang
- b Department of Otolaryngology , Kun Shan Hospital of Traditional Chinese Medicine , Jiangsu Province , P.R. China
| | - Pin Dong
- a Department of Otolaryngology , Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , P.R. China
| | - Bin Shen
- a Department of Otolaryngology , Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , P.R. China
| | - Yu Li
- a Department of Otolaryngology , Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , P.R. China
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35
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Vinayak A, Worley DR, Withrow SJ, Adams DS, Powers BE. Dedifferentiated Chondrosarcoma in the Dog and Cat: A Case Series and Review of the Literature. J Am Anim Hosp Assoc 2017; 54:50-59. [PMID: 29131675 DOI: 10.5326/jaaha-ms-6566] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
This retrospective case series describes seven dogs and one cat diagnosed with dedifferentiated chondrosarcoma, an uncommon, aggressive variant of chondrosarcoma. The purpose of the study is to describe clinical, imaging, and histopathological findings of this tumor. Medical records and the diagnostic laboratory database at Colorado State University from 2000 to 2015 were reviewed and complete medical records were available for the eight animals in this report. Similar to what has been reported in people, poor long-term survival and high metastatic rate, particularly to the lungs, was observed in our case series. A bimorphic pattern on imaging (radiographs, computed tomography, and MRI) consisting of mineralized and nonmineralized areas was seen mirroring the high-grade sarcomatous component adjacent to a low-grade chondroid component seen histopathologically. A review of the human literature including suspected etiology, imaging findings, histopathology, and survival times with various treatment options is presented. This article describes the first reported cases of dedifferentiated chondrosarcoma in the veterinary literature. Early accurate recognition could lead to treatment plans tailored to this variant.
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Affiliation(s)
- Arathi Vinayak
- From the VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California (A.V.); and Colorado State University Veterinary Teaching Hospital, Fort Collins, Colorado (A.V., D.R.W., S.J.W., D.S.A., B.E.P.)
| | - Deanna R Worley
- From the VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California (A.V.); and Colorado State University Veterinary Teaching Hospital, Fort Collins, Colorado (A.V., D.R.W., S.J.W., D.S.A., B.E.P.)
| | - Stephen J Withrow
- From the VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California (A.V.); and Colorado State University Veterinary Teaching Hospital, Fort Collins, Colorado (A.V., D.R.W., S.J.W., D.S.A., B.E.P.)
| | - Dustin S Adams
- From the VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California (A.V.); and Colorado State University Veterinary Teaching Hospital, Fort Collins, Colorado (A.V., D.R.W., S.J.W., D.S.A., B.E.P.)
| | - Barbara E Powers
- From the VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California (A.V.); and Colorado State University Veterinary Teaching Hospital, Fort Collins, Colorado (A.V., D.R.W., S.J.W., D.S.A., B.E.P.)
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Kumar R, Duran C, Amini B, Araujo DM, Wang WL. Periosteal mesenchymal chondrosarcoma of the tibia with multifocal bone metastases: a case report. Skeletal Radiol 2017; 46:995-1000. [PMID: 28352960 DOI: 10.1007/s00256-017-2626-5] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2016] [Revised: 03/01/2017] [Accepted: 03/05/2017] [Indexed: 02/02/2023]
Abstract
Mesenchymal chondrosarcoma of bone is a rare high-grade variant of chondrosarcoma, which typically has central intramedullary location. The tumor is characterized by admixture of highly anaplastic small round malignant cells and islands of mineralized low-grade hyaline cartilage. It is most unusual for this tumor to arise on the surface of a long bone. We describe a patient with periosteal mesenchymal chondrosarcoma that arose at the surface of the right tibia with multifocal bone metastases. Radiographic, CT, MRI, and PET-CT features of this unusual tumor are presented.
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Affiliation(s)
- Rajendra Kumar
- Department of Diagnostic Imaging, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA.
| | - Cihan Duran
- Department of Diagnostic Imaging, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA
| | - Behrang Amini
- Department of Diagnostic Imaging, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA
| | - Dejka M Araujo
- Department of Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA
| | - Wei-Lien Wang
- Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA
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Stemm M, Beck C, Mannem R, Neilson J, Klein MJ. Dedifferentiated chondrosarcoma of bone with prominent rhabdoid component. Ann Diagn Pathol 2017. [DOI: 10.1016/j.anndiagpath.2016.11.004] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
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Magliocca KR, Edgar MA, Corey A, Villari CR. Dedifferentiated chondrosarcoma of the larynx: Radiological, gross, microscopic and clinical features. Ann Diagn Pathol 2017; 30:42-46. [PMID: 28965627 DOI: 10.1016/j.anndiagpath.2017.05.010] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2017] [Accepted: 05/15/2017] [Indexed: 02/04/2023]
Abstract
Laryngeal chondrosarcoma is an uncommon malignancy with a predilection for the cricoid cartilage of adult male patients. Although rare, identification of aggressive chondrosarcoma variants, such as dedifferentiated chondrosarcoma (DDCS) may influence preoperative patient counseling, definitive surgical management, potential implementation of post-operative adjuvant therapy and prognosis. Herein we describe clinical and imaging features of laryngeal DDCS, the unique perspective of fresh and formalin fixed macroscopic examination, a spectrum of histopathologic findings, and detail the full course of the patient's disease.
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Affiliation(s)
- Kelly R Magliocca
- Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA 30308, USA.
| | - Mark A Edgar
- Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA 30308, USA
| | - Amanda Corey
- Department of Radiology and Imaging Sciences, Emory University 1364 Clifton Road, NE Atlanta, GA 30322, USA
| | - Craig R Villari
- Department of Otolaryngology, Emory University Hospital, Atlanta, GA 30308, USA
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Liu C, Xi Y, Li M, Jiao Q, Zhang H, Yang Q, Yao W. Dedifferentiated chondrosarcoma: Radiological features, prognostic factors and survival statistics in 23 patients. PLoS One 2017; 12:e0173665. [PMID: 28301537 PMCID: PMC5354284 DOI: 10.1371/journal.pone.0173665] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2016] [Accepted: 02/25/2017] [Indexed: 01/01/2023] Open
Abstract
Background Dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a poor survival. There are many confusing issues concerning the imaging feature that can facilitate early diagnosis and the factors that might be related to outcomes. Methods Twenty-three patients with dedifferentiated chondrosarcoma confirmed by pathology were retrospectively reviewed from 2008 to 2015. The patients’ clinical information, images from radiographs (n = 17), CT (n = 19), and MRI (n = 17), histological features, treatment and prognosis were analyzed. Results There were 12 males and 11 females, and the mean age was 50.39 years old. Fourteen cases affected the axial bone (pelvis, spine), and 9 cases involved the appendicular bone. Seven (41.17%), 9 (47.36), and 12 (66.66%) lesions showed a biphasic nature on radiograph, CT and MRI, respectively. Of the lesions, 17.39% (4/23) were accompanied by pathological fractures. Histologically, the cartilage component was considered histological Grade1 in 12 patients and Grade 2 in 11 patients. The dedifferentiated component showed features of osteosarcoma in 8 cases, malignant fibrous histiocytoma in3 cases, myofibroblastic sarcoma in 1 case and spindle cell sarcoma in 11cases. Twenty-two cases were treated with surgical resection, and 17 cases achieved adequate (wide or radical) surgical margin. In 8 cases, surgery was combined with adjuvant chemotherapy. The overall median survival time was nine months; 17.4% of patients survived to five years. Conclusion Axial bone location, lung metastasis at diagnosis, inadequate surgical margin, incorrect diagnosis before surgery and pathological fractures was related to poorer outcome. Pre- or postoperative chemotherapy had no definitively effect on improved survival.
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Affiliation(s)
- Chenglei Liu
- Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Yan Xi
- Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Mei Li
- Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Qiong Jiao
- Department of Pathology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Huizhen Zhang
- Department of Pathology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Qingcheng Yang
- Department of Orthopedics, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
| | - Weiwu Yao
- Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, China
- * E-mail:
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40
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Chen S, Wang Y, Su G, Chen B, Lin D. Primary intraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications: a case report and review of the literature. World J Surg Oncol 2016; 14:203. [PMID: 27487949 PMCID: PMC4973031 DOI: 10.1186/s12957-016-0963-9] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2016] [Accepted: 07/25/2016] [Indexed: 11/10/2022] Open
Abstract
Background Mesenchymal chondrosarcoma is a rare malignant tumor arising from bone or soft tissues. Instraspinal dumbbell-shaped mesenchymal chondrosarcoma is even rarer; however, it should not be neglected by clinicians. Case presentation A 26-year-old female was referred to our hospital with a 1.5-month history of sciatic pain and numbness in the left leg. Computed tomography and magnetic resonance imaging scans revealed an intraspinal dumbbell-shaped mass which had distinguishing features of neurogenic tumors, surprisingly, with massive calcifications, and no tumor metastasis was found. Then the patient underwent a total resection of the tumor, and during the operation, we found that the right nerve root of the fifth lumbar almost disappeared. The tumor was diagnosed as mesenchymal chondrosarcoma by histopathological examination after operation. Adjuvant therapies were not performed. However, recurrence of the tumor occurred 5 months later, and she underwent a total resection again combined with radiotherapy after second surgery. Conclusions To the best of our knowledge, this case study presents the first report in literature about primary instraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications, which may provide some evidence for clinical practice. As the clinical symptoms and radiographic findings of mesenchymal chondrosarcoma are usually not specific, clinicians should consider it as a possible case and diagnose it through careful histopathological examination. Sometimes, calcification could be seen in tumors, which may influence or reflect the growth of tumor and disease prognosis. Although prognosis in mesenchymal chondrosarcoma varies from person to person, generally, complete resection, adjuvant therapy, and regular examinations are recommended to perform for patients with mesenchymal chondrosarcoma.
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Affiliation(s)
- Shudong Chen
- Guangdong Provincial Hospital of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou, 510120, China.,Guangzhou University of Chinese Medicine, Guangzhou, China.,Guangdong Provincial Academy of Chinese Medical Sciences, Guangzhou, China
| | - Yufeng Wang
- Guangdong Provincial Hospital of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou, 510120, China.,Guangdong Provincial Academy of Chinese Medical Sciences, Guangzhou, China
| | - Guoyi Su
- Guangdong Provincial Hospital of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou, 510120, China.,Guangdong Provincial Academy of Chinese Medical Sciences, Guangzhou, China
| | - Bolai Chen
- Guangdong Provincial Hospital of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou, 510120, China.,Guangzhou University of Chinese Medicine, Guangzhou, China.,Guangdong Provincial Academy of Chinese Medical Sciences, Guangzhou, China
| | - Dingkun Lin
- Guangdong Provincial Hospital of Chinese Medicine, No. 111 Dade Road, Yuexiu District, Guangzhou, 510120, China. .,Guangzhou University of Chinese Medicine, Guangzhou, China. .,Guangdong Provincial Academy of Chinese Medical Sciences, Guangzhou, China.
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41
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Le HV, Wadhwa R, Theodore P, Mummaneni P. Excision of Thoracic Chondrosarcoma: Case Report and Review of Literature. Cureus 2016; 8:e708. [PMID: 27588229 PMCID: PMC4999149 DOI: 10.7759/cureus.708] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Chondrosarcomas are cartilage-matrix-forming tumors that make up 20-27% of primary malignant bone tumors and are the third most common primary bone malignancy after multiple myelomas and osteosarcomas. Radiographic assessment of this condition includes plain radiography, computed tomography, and magnetic resonance imaging for tumor characterization and delineation of intraosseous and extraosseous involvement. Most chondrosarcomas are refractory to chemotherapy and radiation therapy; therefore, wide en bloc surgical excision offers the best chance for cure. Chondrosarcomas frequently affect the pelvis and upper and lower extremities. In rare instances, the chest wall can be involved, with chondrosarcomas occurring in the ribs, sternum, anterior costosternal junction, and posterior costotransverse junction. In this article, we present a patient with thoracic chondrosarcoma centered at the left T7 costotransverse joint with effacement of the left T7-T8 neuroforamen. We also detail our operative technique of wide en bloc chondrosarcoma excision and review current literature on this topic.
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Affiliation(s)
- Hai V Le
- Orthopedic Surgery, Massachusetts General Hospital ; Orthopaedics, Brigham and Women's Hospital
| | - Rishi Wadhwa
- Department of Neurological Surgery, UCSF Medical Center
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42
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Abstract
Primary cartilage-forming tumors of the bone are frequent entities in the daily work of skeletal radiologists. This article describes the correlation of pathology and radiology in cartilage-forming skeletal tumors, in particular, enchondroma, osteochondroma, periosteal chondromas, chondroblastoma and various forms of chondrosarcoma. After reading, the radiologist should be able to deduce the different patterns of cartilage tumors on radiographs, CT, and MRI from the pathological aspects. Differentiation of enchondroma and chondrosarcoma is a frequent diagnostic challenge. Some imaging parameters, e. g., deep cortical scalloping (more than two thirds of the cortical thickness), cortical destruction, or a soft-tissue mass, are features of a sarcoma. Osteochondromas are bony protrusions with a continuous extension of bone marrow from the parent bone, the host cortical bone runs continuously from the osseous surface of the tumor into the shaft of the osteochondroma and the osteochondroma has a cartilage cap. Chondromyxoid fibromas are well-defined lytic and eccentric lesions of the metaphysis of the long bones, with nonspecific MRI findings. Chondroblastomas have a strong predilection for the epiphysis of long tubular bones and develop an intense perifocal bone marrow edema. Dedifferentiated chondrosarcomas are bimorphic lesions with a low-grade chondrogenic component and a high-grade noncartilaginous component. Most chondrogenic tumors have a predilection with regard to site and age at manifestation.
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43
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The distinction between chondroma and chondrosarcoma using chemical element mass fractions in tumors determined by neutron activation analysis as diagnostic markers. J Radioanal Nucl Chem 2016. [DOI: 10.1007/s10967-016-4810-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
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Fayad LM, Ahlawat S, Khan MS, McCarthy E. Chondrosarcomas of the hands and feet: A case series and systematic review of the literature. Eur J Radiol 2015; 84:2004-12. [DOI: 10.1016/j.ejrad.2015.06.026] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2015] [Revised: 06/19/2015] [Accepted: 06/24/2015] [Indexed: 10/23/2022]
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45
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Chebib I, Hornicek FJ, Bredella MA, Deshpande V, Nielsen GP. Histologic variants of chondrosarcoma. ACTA ACUST UNITED AC 2014. [DOI: 10.1016/j.mpdhp.2014.03.001] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
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46
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47
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Abstract
Heterotopic ossification is a benign process of mature laminar bone formation in the soft tissues. A synonymous term used to describe this pathology in muscle is myositis ossificans. The pathogenesis is unclear, but is likely multifactorial. The basic pathology is thought to be ectopic production of osseous tissue as part of a repair process in response to tissue injury. This report describes a case of heterotopic ossification of the quadratus lumborum muscle as an incidental finding. This case highlights that treatment is based on symptoms and conservative management might be appropriate for the asymptomatic patient.
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Affiliation(s)
- Brie Alport
- College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada
| | - David Horne
- Medical Imaging, College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada
| | - Brent Burbridge
- Medical Imaging, College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada
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48
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Abstract
Chondrosarcoma is a cartilage forming neoplasm, which is the second most common primary malignancy of bone. Clinicians who treat chondrosarcoma patients must determine the grade of the tumor, and must ascertain the likelihood of metastasis. Acral lesions are unlikely to metastasize, regardless of grade, whereas axial, or more proximal lesions are much more likely to metastasize than tumors found in the distal extremities with equivalent histology. Chondrosarcoma is resistant to both chemotherapy and radiation, making wide local excision the only treatment. Local recurrence is frequently seen after intralesional excision, thus wide local excision is sometimes employed despite significant morbidity, even in low-grade lesions. Chondrosarcoma is difficult to treat. The surgeon must balance the risk of significant morbidity with the ability to minimize the chance of local recurrence and maximize the likelihood of long-term survival.
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Affiliation(s)
- Lee R Leddy
- Medical University of South Carolina, Charleston, SC, USA,
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49
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Khoo MMY, Saifuddin A. The role of MRI in image-guided needle biopsy of focal bone and soft tissue neoplasms. Skeletal Radiol 2013; 42:905-15. [PMID: 23644880 DOI: 10.1007/s00256-013-1630-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2012] [Revised: 03/12/2013] [Accepted: 04/14/2013] [Indexed: 02/02/2023]
Abstract
Magnetic resonance imaging (MRI) plays a critical role in the management pathway of both soft tissue and bone neoplasms, from diagnosis through to post-treatment follow-up. There are a wide range of surgical, oncological, and combined treatment regimes but these rely on accurate histopathological diagnosis. This article reviews the role of MRI in the planning of image-guided needle biopsy for suspected soft tissue and bone tumors.
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Affiliation(s)
- M M Y Khoo
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex HA7 4LP, UK.
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50
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Douis H, Saifuddin A. The imaging of cartilaginous bone tumours. II. Chondrosarcoma. Skeletal Radiol 2013; 42:611-26. [PMID: 23053201 DOI: 10.1007/s00256-012-1521-3] [Citation(s) in RCA: 58] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2012] [Revised: 08/27/2012] [Accepted: 08/30/2012] [Indexed: 02/07/2023]
Abstract
Chondrosarcoma is the third most common primary malignant bone tumour. There are various histological subtypes of chondrosarcomas, of which conventional intramedullary chondrosarcoma is by far the most common. Rarer sub-types include clear cell chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Chondrosarcoma is also classified into central, peripheral and periosteal, dependent upon the lesion site, and into primary chondrosarcoma if the lesion arises de novo and secondary chondrosarcoma if the tumour arises in a pre-existing lesion. The various subtypes of chondrosarcoma have characteristic imaging features that may aid diagnosis and may guide biopsy, therefore potentially preventing misdiagnosis. The aim of this article is to provide an overview of the pertinent clinical and imaging findings of the different forms of chondrosarcoma.
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Affiliation(s)
- H Douis
- Department of Radiology, The Royal National Orthopaedic Hospital NHS Trust, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.
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