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Lee DY, Kim YI, Ryu JS, Kim W. Characterization of sacral chordoma and differential diagnosis from other sacral malignancy using [18F]FDG PET/CT. Medicine (Baltimore) 2024; 103:e37678. [PMID: 38579025 PMCID: PMC10994510 DOI: 10.1097/md.0000000000037678] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2023] [Accepted: 03/01/2024] [Indexed: 04/07/2024] Open
Abstract
2-Deoxy-2-[18F]fluoro-D-glucose ([18F]FDG) positron emission tomography (PET)/computed tomography (CT) is known to be a helpful imaging modality for sacral chordoma, but its detailed characteristics have not been fully described. The purpose of our study was to identify the [18F]FDG PET/CT imaging characteristics of sacral chordoma and compare them with other sacral malignancy. This retrospective study included patients who underwent [18F]FDG PET/CT because of a mass involving the sacrum. Investigated visual findings included visual score and distribution, and semiquantitative parameters measured included standardized uptake values (SUVmax, SUVpeak, SUVmean), tumor-to-liver ratio (TLR), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and tumor size. Comparison studies and receiver operating characteristics (ROC) curve analysis were performed to differentiate between sacral chordoma and other sacral malignancy. Ten patients with sacral chordoma were finally included (M:F = 6:4, median age = 67 yr). On [18F]FDG PET/CT, sacral chordomas presented as a mass with minimal-moderate uptake with a usually heterogenous distribution. Compared with 12 patients with other sacral malignancies (M:F = 4:8, median age 42 yr), sacral chordoma showed a significantly lower TLR (median value 2.1 vs 6.3, P = .021). In ROC curve analysis, TLR showed the largest area under the curve (AUC) of 0.79 (cutoff ≤ 4.0; sensitivity 100.0%, specificity 58.3%; P = .004), and SUVmax showed the second largest AUC of 0.73 (cutoff ≤ 6.9; sensitivity 80.0%, specificity 66.7%; P = .034). [18F]FDG PET/CT of sacral chordoma showed minimal-moderate uptake. The TLR of [18F]FDG PET/CT was significantly lower than that of other sacral malignancy and was the most useful parameter for differentiating sacral chordoma, with the largest AUC. SUVmax could be another helpful semiquantitative parameter.
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Affiliation(s)
- Dong Yun Lee
- Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Yong-il Kim
- Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jin-Sook Ryu
- Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Wanlim Kim
- Department of Orthopedic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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2
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Kobayashi Y, Sugawara Y, Takahata H, Shimizu T, Teramoto N, Ueno T, Ninomiya T. Multiple benign notochordal cell tumors in lung with cystic change. Radiol Case Rep 2023; 18:3117-3121. [PMID: 37416319 PMCID: PMC10319637 DOI: 10.1016/j.radcr.2023.06.014] [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: 03/27/2023] [Revised: 06/05/2023] [Accepted: 06/07/2023] [Indexed: 07/08/2023] Open
Abstract
Benign notochordal cell tumor (BNCT) is a benign lesion derived from notochordal cells. Although it is relatively common in intraosseous lesion, pulmonary BNCT is extremely rare. We present a case of 54-year-old male with multiple pulmonary nodules, in which were considered to be metastatic chordomas initially. For 20 months follow-up without any therapy, most of the nodules had no remarkable change but some nodules showed cystic change. We consulted with pathologists specializing in chordoma and the final diagnosis of the nodules was considered as BNCT rather than chordoma. We herein report the case of multiple pulmonary BNCTs with cystic change, comparing with previous reports.
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Affiliation(s)
- Yusuke Kobayashi
- Department of Diagnostic Radiology, National Hospital Organization, Shikoku Cancer Center, Minamiumemotomachi Kou 160, Matsuyama City, Ehime, 791-0245, Japan
| | - Yoshifumi Sugawara
- Department of Diagnostic Radiology, National Hospital Organization, Shikoku Cancer Center, Minamiumemotomachi Kou 160, Matsuyama City, Ehime, 791-0245, Japan
| | - Hiroyuki Takahata
- Department of Pathology, National Hospital Organization, Shikoku Cancer Center, Matsuyama, Japan
| | - Teruhiko Shimizu
- Department of Diagnostic Radiology, National Hospital Organization, Shikoku Cancer Center, Minamiumemotomachi Kou 160, Matsuyama City, Ehime, 791-0245, Japan
| | - Norihiro Teramoto
- Department of Pathology, National Hospital Organization, Shikoku Cancer Center, Matsuyama, Japan
| | - Tsuyoshi Ueno
- Department of Thoracic Surgery, National Hospital Organization, Shikoku Cancer Center, Matsuyama, Japan
| | - Takashi Ninomiya
- Department of Thoracic Oncology, National Hospital Organization Shikoku Cancer Center, Matsuyama, Japan
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3
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Murphey MD, Minn MJ, Contreras AL, Koeller KK, Shih RY, Inwards CY, Yamaguchi T. Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation. Skeletal Radiol 2023; 52:349-363. [PMID: 36063190 DOI: 10.1007/s00256-022-04158-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2022] [Revised: 07/27/2022] [Accepted: 07/29/2022] [Indexed: 02/02/2023]
Abstract
Benign notochordal cell tumor (BNCT) and chordoma are neoplasms of notochordal differentiation. BNCT represents notochordal rests, commonly an incidental lesion present in the spine in 19% of cadaveric specimens. BNCTs are often radiographically occult. CT of BNCT frequently reveals patchy sclerosis between areas of maintained underlying trabeculae. BNCT demonstrates marrow replacement on T1-weighted MR images with high signal intensity on T2-weighting. BNCTs are frequently smaller than 35 mm and lack significant enhancement, bone destruction, cortical permeation, or soft tissue components. Biopsy or surgical resection of BNCT is usually not warranted, although imaging surveillance may be indicated. Chordoma is a rare low-grade locally aggressive malignancy representing 1-4% of primary malignant bone tumors. Chordoma is most frequent between the ages of 50-60 years with a male predilection. Clinical symptoms, while nonspecific and location dependent, include back pain, numbness, myelopathy, and bowel/bladder incontinence. Unfortunately, lesions are often large at presentation owing to diagnosis delay. Imaging of chordoma shows variable mixtures of bone destruction and sclerosis, calcification (50-70% at CT) and large soft tissue components. MR imaging of chordoma reveals multilobulated areas of marrow replacement on T1-weighting and high signal intensity on T2-weighting reflecting the myxoid component within the lesion and areas of hemorrhage seen histologically. Treatment of chordoma is primarily surgical with prognosis related to resection extent. Unfortunately, complete resection is often not possible (21-75%) resulting in high local recurrence incidence (19-75%) and a 5-year survival rate of 45-86%. This article reviews and illustrates the clinical characteristics, pathologic features, imaging appearance spectrum, treatment, and prognosis of BNCT and spinal chordoma.
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Affiliation(s)
- Mark D Murphey
- Musculoskeletal Imaging and Neuroradiology, ACR Institute for Radiologic Pathology (AIRP), 1100 Wayne Avenue, Suite 1020, Silver Spring, MD, 20910, USA. .,Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD, 20814, USA. .,Department of Radiology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave., Bethesda, MD, 20889, USA.
| | - Matthew J Minn
- Musculoskeletal Imaging and Neuroradiology, ACR Institute for Radiologic Pathology (AIRP), 1100 Wayne Avenue, Suite 1020, Silver Spring, MD, 20910, USA.,Department of Radiology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave., Bethesda, MD, 20889, USA.,Department of Radiology, Scripps Green Hospital, 10666 N. Torrey Pines Road, La Jolla, CA, 92037, USA
| | | | - Kelly K Koeller
- Musculoskeletal Imaging and Neuroradiology, ACR Institute for Radiologic Pathology (AIRP), 1100 Wayne Avenue, Suite 1020, Silver Spring, MD, 20910, USA.,Department of Radiology, Head and Neck Cancer Center, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA
| | - Robert Y Shih
- Musculoskeletal Imaging and Neuroradiology, ACR Institute for Radiologic Pathology (AIRP), 1100 Wayne Avenue, Suite 1020, Silver Spring, MD, 20910, USA.,Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD, 20814, USA.,Department of Radiology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave., Bethesda, MD, 20889, USA
| | - Carrie Y Inwards
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA
| | - Takehiko Yamaguchi
- Department of Pathology, Nikko Medical Center, Dokkyo Medical University, 632 Takatoku, Nikko, Tochigi, 321-2593, Japan
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Update of pediatric bone tumors-notochordal tumors, chondrogenic tumors, and vascular tumors of the bone. Skeletal Radiol 2022; 52:1101-1117. [PMID: 36369290 DOI: 10.1007/s00256-022-04235-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2022] [Revised: 11/04/2022] [Accepted: 11/06/2022] [Indexed: 11/12/2022]
Abstract
There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. In the current manuscript, we address notochordal tumors, chondrogenic tumors, and vascular tumors of the bone.
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5
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Camacho M, Carvalho M, Munhoz R, Etchebehere M, Etchebehere E. FDG PET/CT in bone sarcomas. Nucl Med Mol Imaging 2022. [DOI: 10.1016/b978-0-12-822960-6.00062-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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6
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Abstract
This review provides an overview of the spectrum of tumors showing notochordal differentiation. This spectrum encompasses benign entities that are mostly discovered incidentally on imaging, reported as benign notochordal cell tumor, usually not requiring surgical intervention; slowly growing and histologically low-grade tumors referred to as conventional chordoma but associated with a significant metastatic potential and mortality; and more aggressive disease represented by histologically higher-grade tumors including dedifferentiated chordoma, a high-grade biphasic tumor characterized by a conventional chordoma juxtaposed to a high-grade sarcoma, usually with a spindle or pleomorphic cell morphology, and associated with a poor prognosis and poorly differentiated chordoma.
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Affiliation(s)
- Roberto Tirabosco
- Department of Histopathology, 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
| | - Adrienne M Flanagan
- Department of Histopathology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex HA7 4LP, UK; UCL Cancer Institute, University College London, 72 Huntley Street, London WC1 E 6DD, UK
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7
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Chordoma: 18F-FDG PET/CT and MRI imaging features. Skeletal Radiol 2021; 50:1657-1666. [PMID: 33521875 DOI: 10.1007/s00256-021-03723-w] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/25/2020] [Revised: 01/21/2021] [Accepted: 01/21/2021] [Indexed: 02/02/2023]
Abstract
OBJECTIVE Examine the 18F-FDG PET/CT and MRI imaging characteristics of chordoma. MATERIALS AND METHODS Biopsy-proven chordoma with a pre-therapy 18F-FDG PET/CT from 2001 through 2019 in patients > 18 years old were retrospectively reviewed. Multiple PET/CT and MRI imaging parameters were assessed. RESULTS A total of 23 chordoma patients were included (16 M, 7 F; average age of 60.1 ± 13.0 years) with comparative MRI available in 22 cases. This included 13 sacrococcygeal, 9 mobile spine, and one clival lesions. On 18F-FDG PET/CT, chordomas demonstrated an average SUVmax of 5.8 ± 3.7, average metabolic tumor volume (MTV) of 160.2 ± 263.8 cm3, and average total lesion glycolysis (TLG) of 542.6 ± 1210 g. All demonstrated heterogeneous FDG activity. On MRI, chordomas were predominantly T2 hyperintense (22/22) and T1 isointense (18/22), contained small foci of T1 hyperintensity (17/22), and demonstrated heterogeneous enhancement (14/20). There were no statistically significant associations found between 18F-FDG PET/CT and MRI imaging features. There was no relationship of SUVmax (p = 0.53), MTV (p = 0.47), TLG (p = 0.48), maximal dimension (p = 0.92), or volume (p = 0.45) to the development of recurrent or metastatic disease which occurred in 6/22 patients over a mean follow-up duration of 4.1 ± 2.0 years. CONCLUSION On 18F-FDG PET/CT imaging, chordomas demonstrate moderate, heterogeneous FDG uptake. Predominant T2 hyperintensity and small foci of internal increased T1 signal are common on MRI. The inherent FDG avidity of chordomas suggests that 18F-FDG PET/CT may be a useful modality for staging, evaluating treatment response, and assessing for recurrent or metastatic disease.
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8
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Senne J, Nguyen V, Staner D, Stensby JD, Bhat AP. Demystifying Sacral Masses: A Pictorial Review. Indian J Radiol Imaging 2021; 31:185-192. [PMID: 34316126 PMCID: PMC8299490 DOI: 10.1055/s-0041-1729766] [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] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
The sacrum is a triangular shaped bone made up of five fused vertebral bodies. It is composed of bone, cartilage, marrow elements as well as notochord remnants and is a common site for both benign and malignant (primary and secondary) tumors. Familiarity with the imaging features and clinical presentations of sacral bone tumors could be helpful in narrowing the differential diagnosis. Magnetic resonance imaging and computed tomography are the preferred imaging modalities for evaluating sacral masses. This pictorial review will highlight imaging features of common sacral tumors with pathologic correlation. Additionally, this article will review some critical principles and helpful tips to successfully biopsy these lesions.
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Affiliation(s)
- Julie Senne
- Department of Radiology, University of Missouri-Columbia, Columbia, Missouri, United States
| | - Van Nguyen
- Department of Pathology, University of Missouri-Columbia, Columbia, Missouri, United States
| | - Derek Staner
- Department of Radiology, University of Missouri-Columbia, Columbia, Missouri, United States
| | - James D. Stensby
- Department of Radiology, University of Missouri-Columbia, Columbia, Missouri, United States
| | - Ambarish P. Bhat
- Department of Radiology, University of Missouri-Columbia, Columbia, Missouri, United States
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9
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Chetan MR, Lyon PC, Wu F, Phillips R, Cranston D, Gillies MJ, Bojanic S. Role of diffusion-weighted imaging in monitoring treatment response following high-intensity focused ultrasound ablation of recurrent sacral chordoma. Radiol Case Rep 2019; 14:1197-1201. [PMID: 31428215 PMCID: PMC6698304 DOI: 10.1016/j.radcr.2019.07.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2019] [Accepted: 07/12/2019] [Indexed: 11/25/2022] Open
Abstract
Chordoma is the most common malignant tumor of the sacrum and is associated with significant neurologic morbidity. Local recurrence is very common, and the long-term prognosis is poor. High-intensity focused ultrasound (HIFU) is a noninvasive and nonionising ablative therapy that has been successful in treating other tumor types and is being evaluated as a new therapy for sacral chordoma. Contrast-enhanced magnetic resonance imaging is typically used to evaluate tumor perfusion following HIFU; however, its utility is limited in poorly perfused tumors. Diffusion-weighted imaging (DWI) provides tissue contrast based on differences in the diffusion of extracellular water without using gadolinium-based contrast agents. We present novel DWI findings following a planned partial HIFU ablation of a large sacral chordoma which had recurred after radiotherapy. Following HIFU, the treated tumor volume demonstrated loss of restriction on DWI correlating with photopenia on positron emission tomography. This suggests successful ablation and tumor necrosis. This novel finding may provide guidance for sequence selection when evaluating HIFU therapy for sacral chordoma and other tumor types for which contrast-enhanced magnetic resonance imaging may have limited utility.
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Affiliation(s)
- Madhurima R Chetan
- Nuffield Department of Surgical Sciences, University of Oxford, Room 6607, Level 6, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK.,Department of Radiology, Churchill Hospital, Old Road, Oxford, OX3 7LE, UK
| | - Paul C Lyon
- HIFU Unit, Churchill Hospital, Old Road, Oxford, OX3 7LE, UK.,Department of Radiology, Churchill Hospital, Old Road, Oxford, OX3 7LE, UK
| | - Feng Wu
- Nuffield Department of Surgical Sciences, University of Oxford, Room 6607, Level 6, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK.,HIFU Unit, Churchill Hospital, Old Road, Oxford, OX3 7LE, UK
| | - Rachel Phillips
- Department of Radiology, Churchill Hospital, Old Road, Oxford, OX3 7LE, UK
| | - David Cranston
- Nuffield Department of Surgical Sciences, University of Oxford, Room 6607, Level 6, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK
| | - Martin J Gillies
- Nuffield Department of Surgical Sciences, University of Oxford, Room 6607, Level 6, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK.,Department of Neurosurgery, John Radcliffe Hospital, Headley Way, Oxford, UK
| | - Stana Bojanic
- Department of Neurosurgery, John Radcliffe Hospital, Headley Way, Oxford, UK
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10
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Batouli A, Gholamrezanezhad A, Petrov D, Rudkin S, Matcuk G, Jadvar H. Management of Primary Osseous Spinal Tumors with PET. PET Clin 2018; 14:91-101. [PMID: 30420225 DOI: 10.1016/j.cpet.2018.08.002] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
Knowledge of the PET imaging findings of osseous spinal neoplasms is essential, because they are common incidental findings on PET scans done for staging of unrelated primary malignancies. Additionally, PET can help differentiate lesions that are not clearly defined by anatomic modalities alone. PET can also be used for follow-up of aggressive tumors to assess response to treatment, often proving superior to CT or MR imaging alone for this purpose. This review discusses the role of PET/CT and PET/MR imaging in the diagnosis and management of primary benign and malignant osseous tumors of the spine.
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Affiliation(s)
- Ali Batouli
- Department of Radiology, Division of Neuroradiology, Oregon Health and Science University, 8833 Southwest 30th Avenue, Portland, OR 97219, USA.
| | - Ali Gholamrezanezhad
- Department of Radiology, Division of Musculoskeletal Radiology, Keck School of Medicine, University of Southern California, 2250 Alcazar Street, CSC 102, Los Angeles, CA 90033, USA
| | - David Petrov
- Department of Radiology, Allegheny Health Network, 320 East North Avenue, Pittsburgh, PA 15214, USA
| | - Scott Rudkin
- Department of Radiology, Allegheny Health Network, 320 East North Avenue, Pittsburgh, PA 15214, USA
| | - George Matcuk
- Department of Radiology, Division of Musculoskeletal Radiology, Keck School of Medicine, University of Southern California, 2250 Alcazar Street, CSC 102, Los Angeles, CA 90033, USA
| | - Hossein Jadvar
- Department of Radiology, Division of Nuclear Medicine, Keck School of Medicine, University of Southern California, 2250 Alcazar Street, CSC 102, Los Angeles, CA 90033, USA; Department of Radiology, Keck School of Medicine, University of Southern California, 2250 Alcazar Street, CSC 102, Los Angeles, CA 90033, USA
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11
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Gallicchio R, Nardelli A, Pedicini P, Guglielmi G, Storto G. PET/CT and Bone Scintigraphy: Metabolic Results in Musculoskeletal Lesions. CURRENT RADIOLOGY REPORTS 2018. [DOI: 10.1007/s40134-018-0290-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
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12
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Abstract
Chordoma is a rare midline malignant tumor arising from embryonic remnants of the primitive notochord. The base of the skull is the second most common site of disease after the sacrococcygeal region. Intracranial chordoma constitutes about 30-35% of chordoma cases. Metastasis from chordoma is uncommon but if occurs, it tends to spread to the lungs. Cerebrospinal fluid seeding or drop metastasis is very rare. Here we describe a case of a clival chordoma with drop metastases.
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Affiliation(s)
| | - Vijayadwaja Desai
- Department of Pathology, Khoo Teck Puat Hospital, Singapore, Singapore
| | - Lee Lian Chew
- Division of Oncologic Imaging, National Cancer Center Singapore, Singapore, Singapore
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13
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Abstract
Purpose of Review Chordoma are rare tumours of the axial skeleton which occur most often at the base of the skull and in the sacrum. Although chordoma are generally slow-growing lesions, the recurrence rate is high and the location makes it often difficult to treat. Both computed tomography (CT) and magnetic resonance imaging (MRI) are crucial in the initial diagnosis, treatment planning and post-treatment follow-up. Recent Findings Basic MRI and CT characteristics of chordoma were described in the late 1980s and early 1990s. Since then, imaging techniques have evolved with increased resolution and new molecular imaging tools are rapidly evolving. New imaging tools have been developed not only to study anatomy, but also physiologic changes and characterization of tissue and assessment of tumour biology. Recent studies show the uptake of multiple PET tracers in chordoma, which may become an important aspect in the diagnosis, follow-up and personalized therapy. Summary This review gives an overview of skull base chordoma histopathology, classic imaging characteristics, radiomics and state-of-the-art imaging techniques that are now emerging in diagnosis, treatment planning and disease monitoring of skull base chordoma.
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14
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Parosteal extra-axial chordoma of the second metacarpal bone: a case report with literature review. Skeletal Radiol 2018; 47:579-585. [PMID: 29151144 DOI: 10.1007/s00256-017-2818-z] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2016] [Revised: 09/20/2017] [Accepted: 10/31/2017] [Indexed: 02/02/2023]
Abstract
Extra-axial chordoma is a chordoma that occurs in non-axial locations. It is a very rare tumor, with 20 cases reported to date; 14 in bone and six in soft tissue. Of the 14 skeletal extra-axial chordomas, ten were intramedullary and four were intracortical. We report the first case of parosteal extra-axial chordoma arising in the second metacarpal bone, expressing brachyury on immunohistochemical analysis, and describe the pathologic and radiologic findings. We suggest that extra-axial chordoma can occur in parosteal bone lesions or the hand, without features of bone distribution or bone-specific sites.
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15
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Cui F, Su M, Zhang H, Tian R. Humeral metastasis of sacrococcygeal chordoma detected by fluorine-18 fluorodeoxyglucose positron emission tomography-computed tomography: A case report. Radiol Case Rep 2018; 13:449-452. [PMID: 29904494 PMCID: PMC6000026 DOI: 10.1016/j.radcr.2018.01.026] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2017] [Accepted: 01/27/2018] [Indexed: 02/05/2023] Open
Abstract
Chordomas are rare, slow-growing, locally aggressive bone tumors arising from embryonic remnants of the notochord. Distant metastases most commonly involve the lung, liver, axial skeleton, skin, and lymph nodes. Humeral metastases are extremely rare. We report the case of a recurrent chordoma with humeral metastasis, complicated with pathologic fracture. Fluorine-18 fluorodeoxyglucose positron emission tomography-computed tomography revealed multiple hypermetabolic skeletal lesions, corresponding to the symptoms. Our report suggests that positron emission tomography-computed tomography is useful for evaluation of recurrence and distant metastases of chordomas.
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Affiliation(s)
- Futao Cui
- Department of Nuclear Medicine, West China Hospital, Sichuan University, 37 Guoxue Lane, Wuhou District, Chengdu, Sichuan 610000, P.R. China
| | - Minggang Su
- Department of Nuclear Medicine, West China Hospital, Sichuan University, 37 Guoxue Lane, Wuhou District, Chengdu, Sichuan 610000, P.R. China
| | - Han Zhang
- Department of nuclear medicine and molecular imaging, Singapore General Hospital, Outram Road, Singapore 169608, Singapore
| | - Rong Tian
- Department of Nuclear Medicine, West China Hospital, Sichuan University, 37 Guoxue Lane, Wuhou District, Chengdu, Sichuan 610000, P.R. China
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16
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Ishibashi M, Tanabe Y, Fujii S, Ogawa T. Pictorial review of 18F-FDG PET/CT findings in musculoskeletal lesions. Ann Nucl Med 2017; 31:437-453. [PMID: 28585058 DOI: 10.1007/s12149-017-1182-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/03/2017] [Accepted: 05/27/2017] [Indexed: 11/26/2022]
Abstract
We herein reviewed 18F-fluoro-2-deoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) findings in a number of musculoskeletal lesions including malignant tumors, benign tumors, and tumor-like lesions with correlations to other radiographic imaging modalities, and described the diversity of the 18F-FDG PET/CT findings of this entity. Malignant primary musculoskeletal tumors are typically 18F-FDG avid, whereas low-grade malignant tumors show mild uptake. Benign musculoskeletal tumors generally show a faint uptake of 18F-FDG, and tumor-like conditions also display various uptake patterns of 18F-FDG. Although musculoskeletal tumors show various uptakes of 18F-FDG on PET/CT, its addition to morphological imaging modalities such as CT and MRI is useful for the characterization and differentiation of musculoskeletal lesions.
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Affiliation(s)
- Mana Ishibashi
- Division of Radiology, Department of Pathophysiological and Therapeutic Science, Faculty of Medicine, Tottori University, 36-1 Nishicho, Yonago, 683-8504, Japan.
| | - Yoshio Tanabe
- Division of Radiology, Department of Pathophysiological and Therapeutic Science, Faculty of Medicine, Tottori University, 36-1 Nishicho, Yonago, 683-8504, Japan
| | - Shinya Fujii
- Division of Radiology, Department of Pathophysiological and Therapeutic Science, Faculty of Medicine, Tottori University, 36-1 Nishicho, Yonago, 683-8504, Japan
| | - Toshihide Ogawa
- Division of Radiology, Department of Pathophysiological and Therapeutic Science, Faculty of Medicine, Tottori University, 36-1 Nishicho, Yonago, 683-8504, Japan
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17
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68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Metastasizing Chordoma: Comparison With 18F-FDG. Clin Nucl Med 2017; 42:e210-e211. [PMID: 28166150 DOI: 10.1097/rlu.0000000000001576] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Chordoma is a rare slow-growing neoplasm of neuroectodermal origin, which frequently recurs after removal and has the potential to metastasize. We present the case of a 53-year-old man with metastasizing chordoma who underwent F-FDG and Ga-DOTA-TATE PET/CT for restaging of disease and for evaluation of targeted radionuclide therapy potential. On both F-FDG and Ga-DOTA-TATE PET scans, increased tracer accumulation was observed in chordoma metastases. Besides the increased glucose metabolism in chordoma, this case highlights the potential of Ga-DOTA-TATE PET/CT for restaging of chordoma as well the option of targeted radionuclide therapy in this entity.
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Chang C, Chebib I, Torriani M, Bredella M. Osseous metastases of chordoma: imaging and clinical findings. Skeletal Radiol 2017; 46:351-358. [PMID: 28064345 DOI: 10.1007/s00256-016-2566-5] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/11/2016] [Revised: 12/13/2016] [Accepted: 12/29/2016] [Indexed: 02/02/2023]
Abstract
PURPOSE To describe the imaging and clinical characteristics of chordoma osseous metastases (COM). MATERIALS AND METHODS Our study was IRB approved and HIPAA compliant. A retrospective search of our pathology database for pathology-proven COM yielded 15 patients who had undergone MRI, CT, bone scan, and/or FDG-PET/CT. The imaging and clinical features of the COMs were recorded. A control group of age and gender matched chordoma patients without osseous metastasis was evaluated. RESULTS The COM mean maximal dimension was 6.4 ± 4.0 cm. The majority (60%) of patients had one lesion. Extra-osseous soft tissue component was present in 85% and was larger than intra-osseous component in 76%. On MRI the lesions were heterogeneous but predominantly T2 hyperintense with hypointense septae, and with variable enhancement. On CT the lesions were typically destructive or permeative; calcifications were rare. The extent of the soft tissue component was isodense to muscle on CT and therefore better evaluated on MRI. COM was in a body part contiguous to the site of the primary tumor. Compared to the controls, COM patients were more likely to have local recurrence (P = 0.0009) and positive resection margins (P = 0.002). At 1 year, 33% of COM patients were deceased and 13% had progressive metastases. CONCLUSION COM are associated with large extra-osseous soft tissue components, which are better visualized by MRI. They are often located in a body part contiguous to the site of the primary tumor, portend poor prognosis, and are associated with positive resection margins and local recurrence.
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Affiliation(s)
- Connie Chang
- Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, 55 Fruit Street Yawkey 6E, Boston, MA, 02114, USA.
| | - Ivan Chebib
- Department of Pathology, Massachusetts General Hospital, Boston, MA, USA
| | - Martin Torriani
- Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, 55 Fruit Street Yawkey 6E, Boston, MA, 02114, USA
| | - Miriam Bredella
- Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, 55 Fruit Street Yawkey 6E, Boston, MA, 02114, USA
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Rohatgi S, Ramaiya NH, Jagannathan JP, Howard SA, Shinagare AB, Krajewski KM. Metastatic Chordoma: Report of the Two Cases and Review of the Literature. Eurasian J Med 2015; 47:151-4. [PMID: 26180502 DOI: 10.5152/eurasianjmed.2015.52] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2014] [Accepted: 09/07/2014] [Indexed: 11/22/2022] Open
Abstract
Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy.
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Affiliation(s)
- Saurabh Rohatgi
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
| | - Nikhil H Ramaiya
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
| | - Jyothi P Jagannathan
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
| | - Stephanie A Howard
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
| | - Atul B Shinagare
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
| | - Katherine M Krajewski
- Brigham and Women's Hospital/Dana Farber Cancer institute/ Harvard Medical School - Radiology Boston, Massachusetts, USA
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Patel P, Brooks C, Seneviratne A, Hess DA, Séguin CA. Investigating microenvironmental regulation of human chordoma cell behaviour. PLoS One 2014; 9:e115909. [PMID: 25541962 PMCID: PMC4277432 DOI: 10.1371/journal.pone.0115909] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2014] [Accepted: 12/01/2014] [Indexed: 12/30/2022] Open
Abstract
The tumour microenvironment is complex and composed of many different constituents, including matricellular proteins such as connective tissue growth factor (CCN2), and is characterized by gradients in oxygen levels. In various cancers, hypoxia and CCN2 promote stem and progenitor cell properties, and regulate the proliferation, migration and phenotype of cancer cells. Our study was aimed at investigating the effects of hypoxia and CCN2 on chordoma cells, using the human U-CH1 cell line. We demonstrate that under basal conditions, U-CH1 cells express multiple CCN family members including CCN1, CCN2, CCN3 and CCN5. Culture of U-CH1 cells in either hypoxia or in the presence of recombinant CCN2 peptide promoted progenitor cell-like characteristics specific to the notochordal tissue of origin. Specifically, hypoxia induced the most robust increase in progenitor-like characteristics in U-CH1 cells, including increased expression of the notochord-associated markers T, CD24, FOXA1, ACAN and CA12, increased cell growth and tumour-sphere formation, and a decrease in the percentage of vacuolated cells present in the heterogeneous population. Interestingly, the effects of recombinant CCN2 peptide on U-CH1 cells were more pronounced under normoxia than hypoxia, promoting increased expression of CCN1, CCN2, CCN3 and CCN5, the notochord-associated markers SOX5, SOX6, T, CD24, and FOXA1 as well as increased tumour-sphere formation. Overall, this study highlights the importance of multiple factors within the tumour microenvironment and how hypoxia and CCN2 may regulate human chordoma cell behaviour.
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Affiliation(s)
- Priya Patel
- Department of Anatomy and Cell Biology, Schulich School of Medicine & Dentistry, The University of Western Ontario, London, Ontario, Canada
| | - Courtney Brooks
- Department of Physiology and Pharmacology, Schulich School of Medicine & Dentistry, The University of Western Ontario, London, Ontario, Canada
| | - Ayesh Seneviratne
- Department of Physiology and Pharmacology, Schulich School of Medicine & Dentistry, The University of Western Ontario, London, Ontario, Canada
- Robarts Research Institute, The University of Western Ontario, London, Ontario, Canada
| | - David A. Hess
- Department of Physiology and Pharmacology, Schulich School of Medicine & Dentistry, The University of Western Ontario, London, Ontario, Canada
- Robarts Research Institute, The University of Western Ontario, London, Ontario, Canada
| | - Cheryle A. Séguin
- Department of Physiology and Pharmacology, Schulich School of Medicine & Dentistry, The University of Western Ontario, London, Ontario, Canada
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21
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George B, Bresson D, Bouazza S, Froelich S, Mandonnet E, Hamdi S, Orabi M, Polivka M, Cazorla A, Adle-Biassette H, Guichard JP, Duet M, Gayat E, Vallée F, Canova CH, Riet F, Bolle S, Calugaru V, Dendale R, Mazeron JJ, Feuvret L, Boissier E, Vignot S, Puget S, Sainte-Rose C, Beccaria K. [Chordoma]. Neurochirurgie 2014; 60:63-140. [PMID: 24856008 DOI: 10.1016/j.neuchi.2014.02.003] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2013] [Revised: 02/14/2014] [Accepted: 03/11/2014] [Indexed: 12/28/2022]
Abstract
PURPOSES To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature. MATERIALS The series of 136 chordomas treated and followed up over 20 years (1972-2012) in the department of neurosurgery at Lariboisière hospital is reviewed. It includes: 58 chordomas of the skull base, 47 of the craniocervical junction, 23 of the cervical spine and 8 from the lombosacral region. Similarly, 31 chordomas in children (less than 18 years of age), observed in the departments of neurosurgery of les Enfants-Malades and Lariboisière hospitals, are presented. They were observed between 1976 and 2010 and were located intracranially (n=22 including 13 with cervical extension), 4 at the craniocervical junction level and 5 in the cervical spine. METHODS In the entire Lariboisière series and in the different groups of localization, different parameters were analyzed: the delay of diagnosis, of follow-up, of occurrence of metastasis, recurrence and death, the number of primary patients and patients referred to us after progression or recurrence and the number of deaths, recurrences and metastases. The influence of the quality of resection (total, subtotal and partial) on the prognosis is also presented. Kaplan-Meier actuarial curves of overall survival and disease free survival were performed in the entire series, including the different groups of localization based on the following 4 parameters: age, primary and secondary patients, quality of resection and protontherapy. In the pediatric series, a similar analysis was carried-out but was limited by the small number of patients in the subgroups. RESULTS In the Lariboisière series, the mean delay of diagnosis is 10 months and the mean follow-up is 80 months in each group. The delay before recurrence, metastasis and death is always better for the skull base chordomas and worse for those of the craniocervical junction, which have similar results to those of the cervical spine. Similar figures were observed as regards the number of deaths, metastases and recurrences. Quality of resection is the major factor of prognosis with 20.5 % of deaths and 28 % of recurrences after total resection as compared to 52.5 % and 47.5 % after subtotal resection. This is still more obvious in the group of skull base chordomas. Adding protontherapy to a total resection can still improve the results but there is no change after subtotal resection. The actuarial curve of overall survival shows a clear cut in the slope with some chordomas having a fast evolution towards recurrence and death in less than 4 years and others having a long survival of sometimes more than 20 years. Also, age has no influence on the prognosis. In primary patients, disease free survival is better than in secondary patients but not in overall survival. Protontherapy only improves the overall survival in the entire series and in the skull base group. Total resection improves both the overall and disease free survival in each group. Finally, the adjunct of protontherapy after total resection is clearly demonstrated. In the pediatric series, the median follow-up is 5.7 years. Overall survival and disease free survival are respectively 63 % and 54.3 %. Factors of prognosis are the histological type (atypical forms), localization (worse for the cervical spine and better for the clivus) and again it will depend on the quality of resection. CONCLUSIONS Many different pathologies derived from the notochord can be observed: some are remnants, some may be precursors of chordomas and some have similar features but are probably not genuine chordomas. To-day, immuno-histological studies should permit to differentiate them from real chordomas. Improving knowledge of molecular biology raises hopes for complementary treatments but to date the quality of surgical resection is still the main factor of prognosis. Complementary protontherapy seems useful, especially in skull base chordomas, which have better overall results than those of the craniocervical junction and of the cervical spine. However, we are still lacking an intrinsic marker of evolution to differentiate the slow growing chordomas with an indolent evolution from aggressive types leading rapidly to recurrence and death on which more aggressive treatments should be applied.
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Affiliation(s)
- B George
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France.
| | - D Bresson
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - S Bouazza
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - S Froelich
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - E Mandonnet
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - S Hamdi
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - M Orabi
- Service de neurochirurgie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - M Polivka
- Service d'anatomopathologie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - A Cazorla
- Service d'anatomopathologie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - H Adle-Biassette
- Service d'anatomopathologie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - J-P Guichard
- Service de neuroradiologie, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - M Duet
- Service de médecine nucléaire, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - E Gayat
- Service d'anesthésie-réanimation, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - F Vallée
- Service d'anesthésie-réanimation, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France
| | - C-H Canova
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - F Riet
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - S Bolle
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - V Calugaru
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - R Dendale
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - J-J Mazeron
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - L Feuvret
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - E Boissier
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - S Vignot
- Service de radiothérapie et d'oncologie médicale, hôpital de la Salpêtrière, institut Gustave-Roussy, institut Curie, 47-83, boulevard de l'Hôpital, 75013 Paris, France
| | - S Puget
- Service de neurochirurgie, hôpital Necker, 149, rue de Sèvres, 75015 Paris, France
| | - C Sainte-Rose
- Service de neurochirurgie, hôpital Necker, 149, rue de Sèvres, 75015 Paris, France
| | - K Beccaria
- Service de neurochirurgie, hôpital Necker, 149, rue de Sèvres, 75015 Paris, France
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Safari M, Khoshnevisan A. An overview of the role of cancer stem cells in spine tumors with a special focus on chordoma. World J Stem Cells 2014; 6:53-64. [PMID: 24567788 PMCID: PMC3927014 DOI: 10.4252/wjsc.v6.i1.53] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2013] [Revised: 08/31/2013] [Accepted: 11/05/2013] [Indexed: 02/06/2023] Open
Abstract
Primary malignant tumors of the spine are relatively rare, less than 5% of all spinal column tumors. However, these lesions are often among the most difficult to treat and encompass challenging pathologies such as chordoma and a variety of invasive sarcomas. The mechanisms of tumor recurrence after surgical intervention, as well as resistance to radiation and chemotherapy, remain a pervasive and costly problem. Recent evidence has emerged supporting the hypothesis that solid tumors contain a sub-population of cancer cells that possess characteristics normally associated with stem cells. Particularly, the potential for long-term proliferation appears to be restricted to subpopulations of cancer stem cells (CSCs) functionally defined by their capacity to self-renew and give rise to differentiated cells that phenotypically recapitulate the original tumor, thereby causing relapse and patient death. These cancer stem cells present a unique opportunity to better understand the biology of solid tumors in general, as well as targets for future therapeutics. The general objective of the current study is to discuss the fundamental concepts for understanding the role of CSCs with respect to chemoresistance, radioresistance, special cell surface markers, cancer recurrence and metastasis in tumors of the osseous spine. This discussion is followed by a specific review of what is known about the role of CSCs in chordoma, the most common primary malignant osseous tumor of the spine.
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Ochoa-Figueroa M, Martínez-Gimeno E, Allende-Riera A, Cabello-García D, Muñoz-Iglesias J, Cárdenas-Negro C. Role of 18F-FDG PET-CT in the study of sacrococcygeal chordoma. Rev Esp Med Nucl Imagen Mol 2012. [DOI: 10.1016/j.remnie.2012.09.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Jahangiri A, Jian B, Miller L, El-Sayed IH, Aghi MK. Skull base chordomas: clinical features, prognostic factors, and therapeutics. Neurosurg Clin N Am 2012; 24:79-88. [PMID: 23174359 DOI: 10.1016/j.nec.2012.08.007] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
Chordomas of the skull base are one of the rarest intracranial malignancies that arise from ectopic remnants of embryonal notochod. The proximity of many chordomas to neurovascular structures makes gross total resection difficult, and the tendency for recurrence leads to the routine use of adjuvant postoperative radiation. Several surgical approaches are used ranging from extensive craniotomies to minimally invasive endonasal endoscopic approaches. In this review, the histopathology and epidemiology, imaging characteristics, surgical approaches, adjuvant therapies, prognostic factors, and molecular biology of chordomas are described.
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Affiliation(s)
- Arman Jahangiri
- Department of Neurological Surgery, University of California, San Francisco, CA 94143, USA
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Abstract
Although most often back pain is of benign origin, it can occasionally be a harbinger of a more serious spinal condition, including spine neoplasm. Knowledge of the typical clinical history of spinal tumors and an understanding of the innervation of the spine and surrounding supporting structures may allow us to better understand when to pursue advanced imaging in the evaluation of spinal pain syndromes. Many radiologists have divided the differential diagnosis of neoplasms of the spine into compartments. These compartments include the extradural compartment, intradural/extramedullary compartment, and the intramedullary compartment.
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Affiliation(s)
- John T Wald
- Department of Radiology, Division of Neuroradiology, Mayo Clinic, Rochester, MN 55901, USA.
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26
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Mammar H, Kerrou K, Nataf V, Pontvert D, Clemenceau S, Lot G, George B, Polivka M, Mokhtari K, Ferrand R, Feuvret L, Habrand JL, Pouysségur J, Mazure N, Talbot JN. Positron emission tomography/computed tomography imaging of residual skull base chordoma before radiotherapy using fluoromisonidazole and fluorodeoxyglucose: potential consequences for dose painting. Int J Radiat Oncol Biol Phys 2012; 84:681-7. [PMID: 22391104 DOI: 10.1016/j.ijrobp.2011.12.047] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2011] [Revised: 12/06/2011] [Accepted: 12/12/2011] [Indexed: 12/12/2022]
Abstract
PURPOSE To detect the presence of hypoxic tissue, which is known to increase the radioresistant phenotype, by its uptake of fluoromisonidazole (18F) (FMISO) using hybrid positron emission tomography/computed tomography (PET/CT) imaging, and to compare it with the glucose-avid tumor tissue imaged with fluorodeoxyglucose (18F) (FDG), in residual postsurgical skull base chordoma scheduled for radiotherapy. PATIENTS AND METHODS Seven patients with incompletely resected skull base chordomas were planned for high-dose radiotherapy (dose ≥70 Gy). All 7 patients underwent FDG and FMISO PET/CT. Images were analyzed qualitatively by visual examination and semiquantitatively by computing the ratio of the maximal standardized uptake value (SUVmax) of the tumor and cerebellum (T/C R), with delineation of lesions on conventional imaging. RESULTS Of the eight lesion sites imaged with FDG PET/CT, only one was visible, whereas seven of nine lesions were visible on FMISO PET/CT. The median SUVmax in the tumor area was 2.8 g/mL (minimum 2.1; maximum 3.5) for FDG and 0.83 g/mL (minimum 0.3; maximum 1.2) for FMISO. The T/C R values ranged between 0.30 and 0.63 for FDG (median, 0.41) and between 0.75 and 2.20 for FMISO (median,1.59). FMISO T/C R >1 in six lesions suggested the presence of hypoxic tissue. There was no correlation between FMISO and FDG uptake in individual chordomas (r = 0.18, p = 0.7). CONCLUSION FMISO PET/CT enables imaging of the hypoxic component in residual chordomas. In the future, it could help to better define boosted volumes for irradiation and to overcome the radioresistance of these lesions. No relationship was founded between hypoxia and glucose metabolism in these tumors after initial surgery.
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Affiliation(s)
- Hamid Mammar
- Radiation Oncology Department, Antoine Lacassagne Center, Nice, France.
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27
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Ochoa-Figueroa MA, Martínez-Gimeno E, Allende-Riera A, Cabello-García D, Muñoz-Iglesias J, Cárdenas-Negro C. Role of 18F-FDG PET-CT in the study of sacrococcygeal chordoma. Rev Esp Med Nucl Imagen Mol 2011; 31:359-61. [PMID: 23169392 DOI: 10.1016/j.remn.2011.11.001] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2011] [Revised: 10/26/2011] [Accepted: 11/04/2011] [Indexed: 11/25/2022]
Affiliation(s)
- M A Ochoa-Figueroa
- Departamento de Medicina Nuclear, Hospital Universitario Insular de Gran Canaria, Las Palmas de Gran Canaria, Spain.
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Thornton E, Krajewski KM, O'Regan KN, Giardino AA, Jagannathan JP, Ramaiya N. Imaging features of primary and secondary malignant tumours of the sacrum. Br J Radiol 2011; 85:279-86. [PMID: 22167504 DOI: 10.1259/bjr/25247602] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023] Open
Abstract
Malignant tumours of the sacrum may be primary or secondary. While sacral metastases are frequently encountered, a diagnostic dilemma can present when there is a single sacral bone tumour with no history or evidence of malignancy elsewhere in the body. Familiarity with the imaging features and clinical presentations of primary malignant bone tumours is helpful in narrowing the differential. This pictorial review will illustrate with both common and uncommon malignant sacral tumours CT, MRI and positron emission tomography/CT, highlighting the specific features of each.
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Affiliation(s)
- E Thornton
- Department of Imaging, Dana Farber Cancer Institute, Boston, MA 02115, USA.
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29
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Current therapeutic options and novel molecular markers in skull base chordomas. Neurosurg Rev 2011; 35:1-13; discussion 13-4. [DOI: 10.1007/s10143-011-0354-1] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2011] [Revised: 06/01/2011] [Accepted: 07/03/2011] [Indexed: 12/13/2022]
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30
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Kato TA, Tsuda A, Uesaka M, Fujimori A, Kamada T, Tsujii H, Okayasu R. In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions and chemotherapeutic drugs. Radiat Oncol 2011; 6:116. [PMID: 21914223 PMCID: PMC3182904 DOI: 10.1186/1748-717x-6-116] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2011] [Accepted: 09/14/2011] [Indexed: 01/31/2023] Open
Abstract
Background Chordoma, a rare cancer, is usually treated with surgery and/or radiation. However, very limited characterizations of chordoma cells are available due to a minimal availability (only two lines validated by now) and the extremely long doubling time. In order to overcome this situation, we successfully derived a cell line with a shorter doubling time from the first validated chordoma line U-CH1 and obtained invaluable cell biological data. Method After isolating a subpopulation of U-CH1 cells with a short doubling time (U-CH1-N), cell growth, cell cycle distribution, DNA content, chromosome number, p53 status, and cell survival were examined after exposure to X-rays, heavy ions, camptothecin, mitomycin C, cisplatin and bleocin. These data were compared with those of HeLa (cervical cancer) and U87-MG (glioblastoma) cells. Results The cell doubling times for HeLa, U87-MG and U-CH1-N were approximately 18 h, 24 h and 3 days respectively. Heavy ion irradiation resulted in more efficient cell killing than x-rays in all three cell lines. Relative biological effectiveness (RBE) at 10% survival for U-CH1-N was about 2.45 for 70 keV/μm carbon and 3.86 for 200 keV/μm iron ions. Of the four chemicals, bleocin showed the most marked cytotoxic effect on U-CH1-N. Conclusion Our data provide the first comprehensive cellular characterization using cells of chordoma origin and furnish the biological basis for successful clinical results of chordoma treatment by heavy ions.
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Affiliation(s)
- Takamitsu A Kato
- Research Center for Charged Particle Therapy, National Institute of Radiological Sciences, 4-9-1 Anagawa, Inage-ku, Chiba-shi, 263-8555 Japan
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Diaz RJ, Cusimano MD. The biological basis for modern treatment of chordoma. J Neurooncol 2011; 104:411-22. [PMID: 21384217 DOI: 10.1007/s11060-011-0559-8] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2011] [Accepted: 02/21/2011] [Indexed: 01/03/2023]
Abstract
Chordomas are rare malignant tumors arising in bone of the spheno-occiput, sacrum, and vertebral column which can cause neurological deficit. Current management of chordoma involves safe resection followed by radiation therapy. However, surgical resection is often subtotal and chordoma often recurs despite optimal therapy. Despite years of effort, effective adjuvant therapy for denovo, recurrent and metastatic chordoma are absent and 5-year survival is at best 65%. While no chemotherapeutic agent has been demonstrated to be effective against chordoma in vivo, a greater understanding of the genetics and molecular biology of chordoma is opening up avenues of investigation towards the rational development of targeted therapies. Although enthusiasm for the use of already established or new investigational agents will increase with greater understanding of chordoma biology, laboratory studies of these agents are important prior to incorporation into clinical human trials. The authors review the current state of knowledge regarding chordoma and offer insight into potential new therapies for this rare and challenging tumor.
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Affiliation(s)
- Roberto Jose Diaz
- Arthur and Sonia Labatt Brain Tumor Research Centre, The Hospital for Sick Children, Toronto, ON, Canada.
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Bradly DP, Reddy VB, Cochran E, Gattuso P. Comparison of cytological features of myxopapillary ependymomas on crush preparations. Diagn Cytopathol 2009; 37:607-12. [DOI: 10.1002/dc.21094] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
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