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Tanishima T, Kurokawa R, Sone M, Kusumoto M, Abe O. Radiological features of desmoid-type fibromatosis: a two-institution retrospective study. Eur Radiol 2025; 35:1394-1404. [PMID: 39888408 DOI: 10.1007/s00330-024-11285-3] [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: 09/17/2024] [Revised: 10/21/2024] [Accepted: 11/12/2024] [Indexed: 02/01/2025]
Abstract
OBJECTIVES To characterize the radiological findings of desmoid-type fibromatosis (DF). METHODS This two-institution retrospective study included 152 patients with pathologically confirmed DF who underwent computed tomography (CT), magnetic resonance imaging (MRI), or 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT between January 2001 and February 2024. Two board-certified radiologists independently evaluated the CT, MRI, and FDG-PET/CT findings, and a third board-certified radiologist resolved discrepancies. Imaging was performed with and without contrast media: 70 patients underwent plain CT, 95 underwent contrast-enhanced (CE) CT, 115 patients underwent plain MRI examinations, 100 patients underwent CE-MRI, and 11 patients underwent FDG-PET/CT (most patients underwent several modalities). RESULTS The median age of the patients was 40 years, with a female predominance (male, 39.5% vs female, 60.5%). Swelling or palpable mass was the most frequent symptom (78/152, 51.3%). Gross total resection of DF was performed in 57 patients, with a recurrence rate of 38.6% (22/57). Tumors were most frequently observed in the extra-abdominal region (79/152, 51.6%). Characteristic radiological features included intermediate intensity on T2-weighted imaging (112/113, 99.1%), intermediate-to-high intensity on T1-weighted imaging (109/111, 98.2%), substantial enhancement in the late phase on MRI (100/100, 100%), moderate to strong enhancement in the late phase on CT (18/20, 90%), and arterial penetration sign on CE-CT (25/96, 26.0%). The mean apparent diffusion coefficient (ADC) of DFs was 1.46 × 10-3 mm2/s (range, 1.00-2.20). CONCLUSION This study highlights the unique imaging features of DF, including the arterial penetration sign and high mean ADC values, which can aid in differentiating DF from other soft tissue tumors. These findings may improve preoperative diagnostic accuracy and reduce the need for invasive procedures. KEY POINTS Question Imaging findings of DF are not well-documented in large-scale studies. Findings This study identifies unique imaging features of DF, such as the arterial penetration sign and high mean ADC values. Clinical relevance These distinctive imaging characteristics improve diagnostic accuracy for DF and lead to appropriate patient management, as DF requires distinct treatment strategies.
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Affiliation(s)
- Tomoya Tanishima
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
- Department of Diagnostic Radiology, National Cancer Center Hospital, Tokyo, Japan
| | - Ryo Kurokawa
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
| | - Miyuki Sone
- Department of Diagnostic Radiology, National Cancer Center Hospital, Tokyo, Japan
| | - Masahiko Kusumoto
- Department of Diagnostic Radiology, National Cancer Center Hospital, Tokyo, Japan
| | - Osamu Abe
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Tanishima T, Kurokawa R, Sone M, Nakai Y, Kusumoto M. Radiological features of pancreatic desmoid-type fibromatosis: a case series and systematic review. Abdom Radiol (NY) 2025; 50:839-850. [PMID: 39278889 PMCID: PMC11794332 DOI: 10.1007/s00261-024-04570-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2024] [Revised: 08/09/2024] [Accepted: 09/03/2024] [Indexed: 09/18/2024]
Abstract
PURPOSE This retrospective study aimed to investigate the radiological features of pancreatic desmoid-type fibromatosis (PDF) and systematically review the previous publications and two new cases. METHODS We searched PubMed, Cochrane Library, and Web of Science Core Collection and included 31 patients with pathologically proven PDFs with analyzable preoperative computed tomography (CT) and magnetic resonance imaging, including two patients from our institution and 29 patients from 28 publications. Two board-certified radiologists reviewed all images. RESULTS The median age of the patients was 39 years, with a male dominance observed (male, 54.8% vs. female, 45.2%). Abdominal pain was the most frequent symptom, occurring in 58.1% of cases. Surgical resection was performed in all cases of PDFs, resulting in a recurrence rate of 8.3% (2/24). The tumors were most commonly located in the pancreatic tail (23/31, 74.2%). In terms of morphology, a "solid" shape was most prevalent (14/31, 45.2%), followed by a "solid and cystic" shape (9/31, 29.0%) and a "cystic" shape (8/31, 25.8%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT scans (13/20, 65%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), and a presence of cystic components in the tumors (17/31, 54.8%). In 16.1% (5/31) of PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct. CONCLUSION We summarized the clinical and imaging characteristics of PDF. Although the incidence may not be high, cystic components suggesting a dilated pancreatic duct within the tumor are unique imaging features in PDF.
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Affiliation(s)
- Tomoya Tanishima
- Department of Diagnostic Radiology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan
| | - Ryo Kurokawa
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
| | - Miyuki Sone
- Department of Diagnostic Radiology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Yudai Nakai
- Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan
| | - Masahiko Kusumoto
- Department of Diagnostic Radiology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
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Asaad SK, Abdullah AM, Abdalrahman SA, Fattah FH, Tahir SH, Omer CS, Rashid RJ, Hassan MN, Mohammed SH, Kakamad FH, Abdalla BA. Extra‑abdominal recurrent aggressive fibromatosis: A case series and a literature review. Mol Clin Oncol 2023; 19:84. [PMID: 37808248 PMCID: PMC10557105 DOI: 10.3892/mco.2023.2680] [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: 05/20/2023] [Accepted: 08/22/2023] [Indexed: 10/10/2023] Open
Abstract
Aggressive fibromatosis is a rare clonal proliferative tumor arising from mesenchymal cells in the fascia and musculoaponeurotic structures. The aim of the present study was to describe several cases of extra-abdominal recurrent aggressive fibromatosis. The present study was a single-center retrospective case series of patients with recurrent aggressive fibromatosis. The cases were managed at a single private facility. A total of 9 patients with recurrent fibromatosis were included. The mean and median ages of the patients were 29 and 30 years, respectively. In total, two thirds (66.67%) of the cases were female. A negative previous medical history was reported in 7 cases (77.7%), and diabetes and hypertension were reported in 1 case (11.1%). Overall, only 1 case (11.1%) had a family history of breast fibromatosis. The time interval between primary tumor resection and recurrent presentation was 28 months. In 6 cases (66.7%), the tumor was located in the extremities. Pain was the most common presenting symptom in 6 cases (66.7%). All patients had their recurring tumor surgically removed, followed by radiation in 5 cases. The resection margin was positive in 4 cases (44.4%). Each patient was subjected to a careful three-month follow-up for recurrences. On the whole, the present study demonstrates that despite the fact that several therapeutic approaches for extra-abdominal recurrent aggressive fibromatosis have been described in the literature, there is a significant likelihood of recurrence following resection.
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Affiliation(s)
- Saywan K. Asaad
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- College of Medicine, University of Sulaimani, Sulaimani, Kurdistan 46001, Iraq
| | - Ari M. Abdullah
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- Sulaimani Teaching Hospital, Sulaimani, Kurdistan 46001, Iraq
| | | | - Fattah H. Fattah
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- College of Medicine, University of Sulaimani, Sulaimani, Kurdistan 46001, Iraq
| | - Soran H. Tahir
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- College of Medicine, University of Sulaimani, Sulaimani, Kurdistan 46001, Iraq
| | - Choman Sabah Omer
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
| | - Rezheen J. Rashid
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- Department of Radiology, Hiwa Hospital, Sulaimani, Kurdistan 46001, Iraq
| | - Marwan N. Hassan
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- Kscien Organization, Hamdi Str, Azadi Mall, Sulaimani, Kurdistan 46001, Iraq
| | - Shvan H. Mohammed
- Kscien Organization, Hamdi Str, Azadi Mall, Sulaimani, Kurdistan 46001, Iraq
| | - Fahmi H. Kakamad
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- College of Medicine, University of Sulaimani, Sulaimani, Kurdistan 46001, Iraq
- Kscien Organization, Hamdi Str, Azadi Mall, Sulaimani, Kurdistan 46001, Iraq
| | - Berun A. Abdalla
- Department of Scientific Affairs, Smart Health Tower, Sulaimani, Kurdistan 46001, Iraq
- Kscien Organization, Hamdi Str, Azadi Mall, Sulaimani, Kurdistan 46001, Iraq
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Pinto FFE, Mello CAL, Nakagawa SA, Chung WT, Torrezan GT, Barros BDF, Cunha IW, Calsavara VF, Carraro DM, Lopes A. Does the Addition of Mutations of CTNNB1 S45F to Clinical Factors Allow Prediction of Local Recurrence in Patients With a Desmoid Tumor? A Local Recurrence Risk Model. Clin Orthop Relat Res 2023; 481:1978-1989. [PMID: 37104792 PMCID: PMC10499079 DOI: 10.1097/corr.0000000000002627] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/12/2022] [Revised: 01/16/2023] [Accepted: 02/27/2023] [Indexed: 04/29/2023]
Abstract
BACKGROUND The initial approach to the treatment of desmoid tumors has changed from surgical resection to watchful waiting. However, surgery is still sometimes considered for some patients, and it is likely that a few patients would benefit from tumor removal if the likelihood of local recurrence could be predicted. However, to our knowledge, there is no tool that can provide guidance on this for clinicians at the point of care. QUESTION/PURPOSE We sought to explore whether a combined molecular and clinical prognostic model for relapse in patients with desmoid tumors treated with surgery would allow us to identify patients who might do well with surgical excision. METHODS This was a retrospective, single-center study of 107 patients with desmoid tumors who were surgically treated between January 1980 and December 2015, with a median follow-up of 106 months (range 7 to 337 months). We correlated clinical variables (age, tumor size, and localization) and CTNNB1 gene mutations with recurrence-free survival. Recurrence-free survival was estimated using a Kaplan-Meier curve. Univariate and multivariable analyses of time to local recurrence were performed using Cox regression models. A final nomogram model was constructed according to the final fitted Cox model. The predictive performance of the model was evaluated using measures of calibration and discrimination: calibration plot and the Harrell C-statistic, also known as the concordance index, in which values near 0.5 represent a random prediction and values near 1 represent the best model predictions. RESULTS The multivariable analysis showed that S45F mutations (hazard ratio 5.25 [95% confidence interval 2.27 to 12.15]; p < 0.001) and tumor in the extremities (HR 3.15 [95% CI 1.35 to 7.33]; p = 0.008) were associated with a higher risk of local recurrence. Based on these risk factors, we created a model; we observed that patients considered to be at high risk of local recurrence as defined by having one or two factors associated with recurrence (extremity tumors and S45F mutation) had an HR of 8.4 compared with patients who had no such factors (95% CI 2.84 to 24.6; p < 0.001). From these data and based on the multivariable Cox models, we also developed a nomogram to estimate the individual risk of relapse after surgical resection. The model had a concordance index of 0.75, or moderate discrimination. CONCLUSION CTNNB1 S45F mutations combined with other clinical variables are a potential prognostic biomarker associated with the risk of relapse in patients with desmoid tumors. The developed nomogram is simple to use and, if validated, could be incorporated into clinical practice to identify patients at high risk of relapse among patients opting for surgical excision and thus help clinicians and patients in decision-making. A large multicenter study is necessary to validate our model and explore its applicability. LEVEL OF EVIDENCE Level III, therapeutic study.
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Affiliation(s)
- Fabio F. E. Pinto
- Department of Pelvic Surgery, A. C. Camargo Cancer Center, São Paulo, Brazil
| | - Celso A. L. Mello
- Department of Clinical Oncology, A. C. Camargo Cancer Center, São Paulo, Brazil
| | - Suely A. Nakagawa
- Department of Pelvic Surgery, A. C. Camargo Cancer Center, São Paulo, Brazil
| | - Wu Tu Chung
- Department of Pelvic Surgery, A. C. Camargo Cancer Center, São Paulo, Brazil
| | - Giovana T. Torrezan
- Clinical and Functional Genomics Group, International Research Center/CIPE, A. C. Camargo Cancer Center, São Paulo, Brazil
- National Institute of Science and Technology in Oncogenomics and Therapeutic Innovation, São Paulo, Brazil
| | - Bruna D. F. Barros
- Clinical and Functional Genomics Group, International Research Center/CIPE, A. C. Camargo Cancer Center, São Paulo, Brazil
| | - Isabela W. Cunha
- Department of Anatomic Pathology, A. C. Camargo Cancer Center, São Paulo, SP, Brazil
| | - Vinícius F. Calsavara
- Cedars Sinai Medical Center, Samuel Oschin Comprehensive Cancer Institute, Los Angeles, CA, USA
| | - Dirce M. Carraro
- Clinical and Functional Genomics Group, International Research Center/CIPE, A. C. Camargo Cancer Center, São Paulo, Brazil
- National Institute of Science and Technology in Oncogenomics and Therapeutic Innovation, São Paulo, Brazil
| | - Ademar Lopes
- Department of Pelvic Surgery, A. C. Camargo Cancer Center, São Paulo, Brazil
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Wrolstad K, Mischke JJ, Elias AR. Desmoid fibromatosis presenting as lateral hip pain in an outpatient physical therapy clinic: A case report. Physiother Theory Pract 2023; 39:219-226. [PMID: 34823424 DOI: 10.1080/09593985.2021.2008566] [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: 12/16/2022]
Abstract
CASE DESCRIPTION A 21-year-old healthy female presented with severe left lateral hip pain beginning suddenly two weeks prior. Physical examination revealed zero degrees of left hip external rotation passive range of motion with a firm end feel and pain severity and irritability out of proportion to an expected musculoskeletal presentation. She was referred to her physician with a recommendation for imaging to determine the source of pain and appropriateness of physical therapy. OUTCOME Magnetic resonance imaging revealed a foreign mass in her left gluteus medius muscle which biopsy revealed to be a desmoid fibromatosis. CONCLUSION This case demonstrates a thorough differential diagnostic process leading to medical imaging referral in a patient with a non-musculoskeletal source of pain. Physical therapists must be diligent in their differential diagnostic process to ensure appropriateness of their treatments or the need for referral.
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Affiliation(s)
- Kelli Wrolstad
- Physical Therapy Department, University of Montana Physical Therapy, 32 Campus Dr, Missoula, MT, USA
| | - John J Mischke
- Physical Therapy Department, University of Montana Physical Therapy, 32 Campus Dr, Missoula, MT, USA
| | - Audrey Rc Elias
- Physical Therapy Department, University of Montana Physical Therapy, 32 Campus Dr, Missoula, MT, USA
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Zargarbashi R, Mahmoudi E, Khalaj K, Vosoughi F, Mollaeian M, Baghbani S. Periscapular Fibromatosis With Intrathoracic Invasion in a 3-Year-Old Boy. Orthopedics 2022; 45:e276-e279. [PMID: 35700428 DOI: 10.3928/01477447-20220608-05] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Fibromatosis is an extremely rare tumor that arises from the myofascial tissue and has high rates of infiltration and recurrence. Fibromatosis of the chest wall is even less common, and wide surgical resection is the preferred treatment alternative to radiation therapy, chemotherapy, and systemic treatments. We report the case of a 3-year-old boy with radiologically and pathologically confirmed fibromatosis of the periscapular region who underwent wide resection. We discuss diagnosis, treatment options, and technical pearls for a desmoid tumor of the chest wall. The resection should go through healthy tissue because of the infiltrative nature of the tumor. Although visceral involvement did not occur in this case, surgeons should be aware of its possibility, and surgery should be performed by a multidisciplinary team, including a pediatric orthopedic surgeon, a thoracic surgeon, and an anesthesiologist. [Orthopedics. 2022;45(5):e276-e279.].
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Nathenson MJ, Hu J, Ratan R, Somaiah N, Hsu R, DeMaria PJ, Catoe HW, Pang A, Subhawong TK, Amini B, Sweet K, Feister K, Malik K, Jagannathan J, Braschi-Amirfarzan M, Sheren J, Caldas Y, Moreno Tellez C, Rosenberg AE, Lazar AJ, Maki RG, Benedetto P, Cohen J, Trent JC, Ravi V, Patel S, Wilky BA. Systemic Chemotherapies Retain Antitumor Activity in Desmoid Tumors Independent of Specific Mutations in CTNNB1 or APC: A Multi-institutional Retrospective Study. Clin Cancer Res 2022; 28:4092-4104. [PMID: 35180772 PMCID: PMC9475245 DOI: 10.1158/1078-0432.ccr-21-4504] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2021] [Revised: 01/23/2022] [Accepted: 02/16/2022] [Indexed: 01/07/2023]
Abstract
PURPOSE Determine whether specific CTNNB1 or APC mutations in patients with desmoid tumor were associated with differences in clinical responses to systemic treatments. EXPERIMENTAL DESIGN We established a multi-institutional dataset of previously treated patients with desmoid tumor across four U.S. sarcoma centers, including demographic and clinicopathologic characteristics, treatment regimens, and clinical and radiographic responses. CTNNB1 or APC mutation status was determined from prior pathology records, or archival tissue was requested and analyzed by Sanger sequencing and/or next-generation sequencing. Evaluable patients with mutation results were analyzed to determine clinical progression-free survival (cPFS), RECIST 1.1 PFS (rPFS), time to next treatment (TTNT), and overall survival (OS). Kaplan-Meier analysis and Cox proportional hazards regression were performed to identify differences in cPFS, rPFS, TTNT, and OS by mutation subtype, desmoid tumor location, and treatment regimen. RESULTS A total of 259 evaluable patients were analyzed for at least one of the survival outcomes, with 177 patients having mutation data. First- and second-line cPFS, rPFS, and TTNT were not significantly affected by mutation subtype; however, APC-mutant desmoid tumors demonstrated nonstatistically significant inferior outcomes. Extremity/trunk desmoid tumor location and treatment with doxorubicin-based, methotrexate/vinca alkaloids and sorafenib regimens were associated with better clinical outcomes compared with surgery or "other" therapies, including estrogen-receptor blockade and imatinib. OS was significantly worse with APC or CTNNB1 negative/other mutations. CONCLUSIONS Mutation subtype did not affect responses to specific systemic therapies. APC mutations and nonextremity desmoid tumor locations remain prognostic for worse outcomes, and earlier initiation of systemic therapy for these higher-risk desmoid tumors should be prospectively evaluated. See related commentary by Greene and Van Tine, p. 3911.
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Affiliation(s)
- Michael J. Nathenson
- Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts
| | - Junxiao Hu
- Department of Biostatistics, University of Colorado Anschutz Medical Campus, Aurora, Colorado
| | - Ravin Ratan
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Neeta Somaiah
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Robert Hsu
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Peter J. DeMaria
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Heath W. Catoe
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Angela Pang
- Department of Medicine, Mount Sinai School of Medicine, New York, New York
| | - Ty K. Subhawong
- Department of Radiology, University of Miami School of Medicine, Miami, Florida
| | - Behrang Amini
- Department of Musculoskeletal Imaging, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Kevin Sweet
- Department of Radiology, University of Miami School of Medicine, Miami, Florida
| | - Katharina Feister
- Department of Radiology, University of Miami School of Medicine, Miami, Florida
| | - Karan Malik
- Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts
| | - Jyothi Jagannathan
- Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts
| | - Marta Braschi-Amirfarzan
- Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts
| | - Jamie Sheren
- Department of Pathology, University of Colorado School of Medicine, Aurora, Colorado
| | - Yupanqui Caldas
- Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado
| | - Cristiam Moreno Tellez
- Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado
| | - Andrew E. Rosenberg
- Department of Pathology, University of Miami School of Medicine, Miami, Florida
| | - Alexander J. Lazar
- Department of Pathology and Genomic Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Robert G. Maki
- Department of Medicine, Mount Sinai School of Medicine, New York, New York
| | - Pasquale Benedetto
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Jonathan Cohen
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Jonathan C. Trent
- Department of Medicine, University of Miami School of Medicine, Miami, Florida
| | - Vinod Ravi
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Shreyaskumar Patel
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Breelyn A. Wilky
- Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado
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Pandrowala S, Jones RL, Gupta S, Gulia A. Desmoid fibromatosis: is the current picture changing? Future Oncol 2021; 17:3397-3408. [PMID: 34227399 DOI: 10.2217/fon-2021-0003] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Desmoid fibromatosis is a locally aggressive tumor with an unpredictable clinical course. Surgery was once the mainstay of treatment, but the treatment protocol has been constantly evolving and currently active surveillance is the front-line approach. There have been significant insights into the molecular biology with the addition of mutational analysis of CTNNB1 adding to prognostic information. We present a review of the literature with current practice guidelines, also including novel therapeutic targets and ongoing clinical trials, to unravel the next step in the management of sporadic desmoid fibromatosis.
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Affiliation(s)
- Saneya Pandrowala
- Department of Surgical Oncology, Bone & Soft Tissue Service, Tata Memorial Hospital and Homi Bhabha National Institute (HBNI), Mumbai, 400012, India
| | - Robin L Jones
- Department of Medicine, Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK
| | - Sanjay Gupta
- Department of Musculoskeletal Oncology, Glasgow Royal Infirmary, Scotland, UK
| | - Ashish Gulia
- Department of Surgical Oncology, Bone & Soft Tissue Service, Tata Memorial Hospital and Homi Bhabha National Institute (HBNI), Mumbai, 400012, India
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Wang K, Zhang J, Dong M. Clinical features and long-term outcomes of aggressive fibromatosis of the abdominal wall after surgical resection: A retrospective study and literature review. Asia Pac J Clin Oncol 2021; 18:127-132. [PMID: 33629526 DOI: 10.1111/ajco.13525] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Accepted: 11/01/2020] [Indexed: 11/30/2022]
Abstract
AIM Aggressive fibromatosis of the abdominal wall is easily misdiagnosed prior to surgery as abdominal wall endometriosis or other tumors. The authors intend to evaluate the clinical features and outcomes of aggressive fibromatosis of the abdominal wall after surgical resection. METHODS We retrospectively analyzed the data from 24 patients treated in the First Hospital of China Medical University from January 2011 to June 2019 and discussed the characteristic of this disease after a review of literature worldwide. RESULTS Most patients were female (91.7%) with a mean age of 33 years (range 26-56 years). The most common symptom was progressive abdominal wall discomfort (ie, abdominal pain) in 16 patients (66.7%). Twelve patients (50%) had a history of cesarean section. All 24 patients underwent surgical R0 resection due to progression of disease or pain, including 12 patients treated with simple tumor resection and 12 patients treated with tumor resection followed by abdominal wall tension-free repair using synthetic mesh. Postoperative complications included incisional infection in three patients (12.5%), incisional hernia and postoperative bleeding in one patient each (4.2%). The local recurrence rate was 12.5%. CONCLUSIONS Aggressive fibromatosis of the abdominal wall mainly occurs in young women after cesarean section or other surgeries, with a high recurrence rate. Radical resection is an optimal treatment for the patients with progressive cases, and the restoration of extensive defects of the abdominal wall can be achieved with synthetic mesh, which leads to a good restoration of abdominal wall integrity.
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Affiliation(s)
- Kewei Wang
- Department of Gastrointestinal Surgery, the First Affiliated Hospital of China Medical University, Shenyang, Liaoning, China
| | - Jiaqi Zhang
- Department of Gastrointestinal Surgery, the First Affiliated Hospital of China Medical University, Shenyang, Liaoning, China
| | - Ming Dong
- Department of Gastrointestinal Surgery, the First Affiliated Hospital of China Medical University, Shenyang, Liaoning, China
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Zhou S, Tang F, Min L, Luo Y, Zhou Y, Duan H, Tu C. [Long-term effectiveness of "West China Classification" guided surgical treatment of desmoid-type fibromatosis in shoulder girdle]. ZHONGGUO XIU FU CHONG JIAN WAI KE ZA ZHI = ZHONGGUO XIUFU CHONGJIAN WAIKE ZAZHI = CHINESE JOURNAL OF REPARATIVE AND RECONSTRUCTIVE SURGERY 2020; 34:744-750. [PMID: 32538566 DOI: 10.7507/1002-1892.201912143] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Objective To evaluate the long-term effectiveness of patients received surgical treatment under the guidance of "West China Classification" of desmoid-type fibromatosis (DTF) in the shoulder girdle. Methods The clinical data of 32 patients with DTF in the shoulder girdle admitted between June 2003 and December 2016 were retrospectively analyzed, including 14 males and 18 females, aged 14-56 years with an average age of 36.8 years. The maximum diameter of the tumor was 7-19 cm, with an average of 11.1 cm. According to the "West China Classification" of DTF in the shoulder girdle, there were 4 cases of region Ⅰ, 3 cases of region Ⅱ, 6 cases of region Ⅲ, 3 cases of region Ⅳ, 5 cases of regions Ⅰ+Ⅱ, 5 cases of regions Ⅱ+Ⅲ, and 6 cases of regions Ⅰ+Ⅱ+Ⅲ. In addition, the involvement of blood vessels and nerves was also taken into consideration for choosing a surgical approach. Finally, 12 cases were operated via anteroposterior approach (group A), 14 via posterior approach (group B), and 6 via combined anterior-posterior approach (group C). The 1993 Musculoskeletal Tumor Society (MSTS93) score (including pain, limb function, satisfaction, hand position, hand flexibility, and lifting ability), Japanese Orthopedic Association (JOA) score, range of motion (ROM) of shoulder joint (including flexion, extension, abduction, and adduction), and complications of patients in the 3 groups were recorded and compared. Results All the 32 patients were followed up 30-190 months, with an average of 94.6 months. At last follow-up, complications occurred in 5 cases (15.6%), including 2 cases (16.6%) in group A, 2 (14.3%) in group B, and 1 (16.6%) in group C. There was no significant difference in the incidence of complications among the 3 groups ( P=1.000). Tumor recurrence occurred in 5 (15.6%) cases, including 1 (8.3%) case in group A, 2 (14.3%) in group B, and 1 (16.6%) in group C. No significant difference was found in the recurrence rate among the 3 groups ( P=1.000). At last follow-up, MSTS93 score of pain, limb function, satisfaction, hand flexibility, and hand position in groups A and B were significantly better than those in group C ( P<0.05), even though no significant difference existed between group A and group B ( P>0.05). The lifting ability score in group C was significantly lower than in group A ( P<0.05), and no significant difference was found between other groups ( P>0.05). The JOA score and flexion, extension, abduction, and adduction activities of shoulder in groups A and B were significantly better than those in group C ( P<0.05). The extension activity in group A was significantly better than that in group B ( P<0.05), the flexion activity in group B was significantly better than that in group A ( P<0.05). There was no significant difference in other indexes between groups A and B ( P>0.05). Conclusion Taking a rational approach to fully expose and completely remove the tumor is the key point of surgical treatment for patients with DTF in the shoulder girdle. At the same time, preservation of vital structures and reconstruction of soft tissues should also be taken into consideration. Overall, surgical treatment under the guidance of "West China Classification" of DTF in the shoulder girdle has achieved satisfactory long-term effectiveness.
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Affiliation(s)
- Sisi Zhou
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Fan Tang
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Li Min
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Yi Luo
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Yong Zhou
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Hong Duan
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
| | - Chongqi Tu
- Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P.R.China
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11
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Kim Y, Rosario MS, Cho HS, Han I. Factors Associated with Disease Stabilization of Desmoid-Type Fibromatosis. Clin Orthop Surg 2020; 12:113-119. [PMID: 32117547 PMCID: PMC7031434 DOI: 10.4055/cios.2020.12.1.113] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2018] [Accepted: 07/18/2019] [Indexed: 11/06/2022] Open
Abstract
Background Spontaneous disease stabilization of desmoid-type fibromatosis (DF) has been demonstrated in many reports, and the watchful waiting approach without any frontline treatment is becoming popular as an initial management strategy. In this study, we aimed to assess the disease stabilization rate and identify predictive factors for disease stabilization of DF in patients with conservative treatment. Methods We reviewed 76 patients with sporadic extra-abdominal DF who were managed with frontline conservative treatment in our institute. The minimum follow-up was 12 months. Stabilization was defined as radiological evidence of no change or continuous decrease in size of the tumor for six months or more. The primary endpoint was stabilization of DF. Possible patient-, disease-, and treatment-related factors predictive of disease stabilization were analyzed with multivariate analysis. Results At final follow-up, 54 of the 76 tumors (71%) were stable, and mean time to stabilization was 30.4 months (range, 7 to 112 months). On Kaplan-Meier survival analysis, the spontaneous stabilization rate was 25.4% at one year, 52.7% at two years, and 70.9% at three years. The mean time to spontaneous stabilization was longer in patients with ≤ 40 years of age (p = 0.022) or recurrence (p = 0.041). On multivariate analysis with the Cox proportional hazard method, recurrence (hazard ratio [HR], 1.79; p = 0.041) and younger age (HR, 2.04; p = 0.022) were identified as independent prognostic factors for longer time to disease stabilization. Conclusions Frontline conservative treatment seems to be the optimal treatment for most patients with DF. Younger patients or those with recurrence may require longer time to spontaneous disease stabilization.
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Affiliation(s)
- Yongsung Kim
- Musculoskeletal Tumor Center, Seoul National University Cancer Hospital, Seoul, Korea
| | - Mamer S Rosario
- Musculoskeletal Tumor Center, Seoul National University Cancer Hospital, Seoul, Korea.,East Avenue Medical Center, Quezon City, Philippines
| | - Hwan Seong Cho
- Department of Orthopaedic Surgery, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Ilkyu Han
- Musculoskeletal Tumor Center, Seoul National University Cancer Hospital, Seoul, Korea.,Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Korea
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12
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Kito M, Ogose A, Yoshida M, Nishida Y. Usefulness of surgical treatment for asymptomatic patients with extra-peritoneal desmoid-type fibromatosis: a systematic review and meta-analysis. Jpn J Clin Oncol 2020; 50:574-580. [DOI: 10.1093/jjco/hyaa009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2019] [Revised: 12/26/2019] [Accepted: 01/14/2020] [Indexed: 12/20/2022] Open
Abstract
Abstract
Objective
The purpose of this systematic review is to assess and compare the efficacy of surgical treatment for patients with asymptomatic extra-peritoneal desmoid-type fibromatosis to the wait-and-see policy by evaluating (1) the exacerbation rate (exacerbation; recurrence after surgery or progressive disease following non-surgical treatment) and (2) treatment-associated complications in extra-peritoneal desmoid-type fibromatosis.
Methods
We evaluated documents published between 1 January 1990 and 31 August 2017. The risk of bias in the selected literature was analyzed using the Cochrane Collaboration Risk of Bias Tool. Quality of evidence was evaluated using Grading of Recommendation, Assessment, Development and Evaluation approach.
Results
One prospective cohort study, four case–control studies and five case series studies were identified. Meta-analysis was performed to evaluate the exacerbation rate after treatment on one prospective cohort study and four case–control studies. In comparing surgical and non-surgical treatments, the exacerbation rate was significantly higher in the surgical treatment group (odds ratio: 1.32, 95% confidence interval 1.01–1.73, P = 0.05). However, in the case series study, the recurrence rate was 23.4% for the surgical treatment group, while the progressive disease rate was 28.1% for the non-surgical treatment group. The postoperative complication rates associated with surgical treatment in the two studies were 20.8 and 17.2%, respectively.
Conclusions
When considering the exacerbation rate, non-surgical treatment might be appropriate for asymptomatic patients with extra-peritoneal desmoid-type fibromatosis. However, if patients with tumor-related symptoms opt for surgery, including those who face difficulties due to the presence of tumors, it is important to fully explain to them the possibility that the recurrence rate and treatment-associated functional failures may increase depending on the site of occurrence.
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Affiliation(s)
- Munehisa Kito
- Department of Orthopaedic Surgery, Shinshu University School of Medicine, Matsumoto, Japan
| | - Akira Ogose
- Department of Orthopaedic Surgery, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Niigata, Japan
| | - Masahiro Yoshida
- Department of Hemodialysis and Surgery, Ichikawa Hospital, International University of Health and Welfare, Chiba, Japan
- Department of EBM and Guidelines, Japan Council for Quality Health Care, Tokyo, Japan, and
| | - Yoshihiro Nishida
- Department of Rehabilitation Medicine, Nagoya University Hospital, Nagoya, Japan
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13
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Duhil de Bénazé G, Vigan M, Corradini N, Minard-Colin V, Marie-Cardine A, Verite C, Defachelles AS, Thebaud E, Castex MP, Sirvent N, Bodet D, Mansuy L, Rome A, Petit A, Plantaz D, Jourdain A, Mary P, Carton M, Orbach D. Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life. Eur J Surg Oncol 2020; 46:1294-1300. [PMID: 32173177 DOI: 10.1016/j.ejso.2020.02.028] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2019] [Revised: 02/11/2020] [Accepted: 02/20/2020] [Indexed: 12/19/2022] Open
Abstract
BACKGROUND With recent conservative strategies, prognosis of patients with desmoid-type fibromatosis (DTF) is about function preservation. We analyzed the long-term quality of life (QoL) of pediatric patients with DTF. METHODS All French young patients (<21years) treated between 2005 and 2016 for a DTF in the EpSSG NRSTS-05 study were analyzed. A first wait-and-see strategy was recommended. Patients' QoL was analyzed with the internationally validated Child Health Questionnaire (CHQ). We focused on the relevant subscales scores: physical functioning (PF), role social limitations physical (RP), bodily pain (BP), general health perception (GH) and physical (PhS) and psychosocial (PsS) summary measures. RESULTS Among the 81 patients, 52 families answered the CHQ (median delay since diagnosis = 6.2years; min2.2-max13.3 years). Median age at diagnosis was 11.5 years. Primary site: limbs (52%), head/neck (27%), or trunk (21%). Five year-Progression Free Survival was 39.1% (95%CI: 27.7-50.5%). As initial management for these 52 patients, 30 patients were first observed (57%), 13 had surgery (25%) and 9 received chemotherapy (18%). Total burden of therapy was exclusive surgery (9pts/18%), exclusive chemotherapy (18pts/35%), surgery + chemotherapy (13pts/25%), chemotherapy + radiotherapy (1 pt), surgery + chemotherapy + radiotherapy (1 pt), wait and see (10 pt). Regarding the parent forms, patients have significant lower PF (86.0vs.96.1; p = 0.03), RP (82.0vs.93.6; p = 0.04), GH (60vs.73; p < 0.005) and PhS (46.2 vs.53; p = 0.02) scores compared to healthy population. Comparison of QoL subscales scores according to initial strategy (wait-and-see vs.surgery/chemotherapy) did not reveal any difference (PF = 87.3vs.84.9; p = 0.80/RP = 83.4vs.78.7; p = 0.72/BP = 78.9vs.78.2; p = 0.95/GH = 59.7vs60; p = 0.97). Similar results were found using the children or adult forms. CONCLUSIONS Initial wait-and-see strategy does not affect long term functional impairment.
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Affiliation(s)
- G Duhil de Bénazé
- University Hospital of Nice- Archet 2, Department of Pediatric Hematology-Oncology, Nice, France; Institut Curie, SIREDO Oncology Center Care- Innovation and Research for Children and AYA with Cancer- PSL Research University, Paris, France.
| | - M Vigan
- Institut Curie- PSL Research University, Biometry Unit, Paris, France
| | - N Corradini
- IHOPe, Centre Régional de Lutte Contre le Cancer- Centre Léon Bérard, Lyon, France
| | - V Minard-Colin
- Gustave Roussy Cancer Campus, Department of Children and Adolescents Oncology, Villejuif, France
| | - A Marie-Cardine
- Rouen University Hospital, Department of Pediatric Hematology-Oncology, Rouen, France
| | - C Verite
- Bordeaux University Hospital, Pediatric Hematology Department, Bordeaux, France
| | - A S Defachelles
- Centre Oscar Lambret, Service D'Oncologie Pédiatrique, Lille, France
| | - E Thebaud
- University Hospital Nantes, Pediatric Oncology Department, Nantes, France
| | - M P Castex
- Children's Hospital of Toulouse- CHU Toulouse, Pediatric Hemato-oncology Department, Toulouse, France
| | - N Sirvent
- University Hospital of Montpellier, Department of Pediatric Onco-Hematology, Montpellier, France
| | - D Bodet
- Caen University Hospital, Department of Pediatric Hematology and Oncology, Caen, France
| | - L Mansuy
- Children's University Hospital, Department of Pediatric Hematology and Oncology, Nancy, France
| | - A Rome
- Centre Hospitalier Universitaire, Department of Pediatric Oncology, Marseille, France
| | - A Petit
- Assistance Publique-Hôpitaux de Paris AP-HP- GH HUEP- Armand Trousseau Hospital, Department of Pediatric Hematology and Oncology, Paris, France
| | - D Plantaz
- University Hospital Centre of Grenoble, Department of Pediatric Hematology-Oncology, Grenoble, France
| | - A Jourdain
- CHU Tours, Department of Pediatric Oncology and Hematology, Tours, France
| | - P Mary
- Pediatric Orthopedics Department, Armand Trousseau Hospital, Paris, France
| | - M Carton
- Institut Curie- PSL Research University, Biometry Unit, Paris, France
| | - D Orbach
- Institut Curie, SIREDO Oncology Center Care- Innovation and Research for Children and AYA with Cancer- PSL Research University, Paris, France
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14
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Cai HJ, Wang H, Cao N, Wang W, Sun XX, Huang B. Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature. World J Clin Cases 2020; 8:577-586. [PMID: 32110669 PMCID: PMC7031834 DOI: 10.12998/wjcc.v8.i3.577] [Citation(s) in RCA: 3] [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: 10/12/2019] [Revised: 01/07/2020] [Accepted: 01/11/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Peutz-Jeghers syndrome (PJS) and mesenteric fibromatosis (MF) are rare diseases, and PJS accompanying MF has not been previously reported. Here, we report a case of a 36-year-old man with both PJS and MF, who underwent total colectomy and MF surgical excision without regular follow-up. Two years later, he sought treatment for recurrent acute abdominal pain. Emergency computed tomography showed multiple soft tissue masses in the abdominal and pelvic cavity, and adhesions in the small bowel and peritoneum. Partial intestinal resection and excision of the recurrent MF were performed to relieve the symptoms.
CASE SUMMARY A 36-year-old male patient underwent total colectomy for PJS with MF. No regular reexamination was performed after the operation. Two years later, due to intestinal obstruction caused by MF enveloping part of the small intestine and peritoneum, the patient came to our hospital for treatment. Extensive recurrence was observed in the abdomen and pelvic cavity. The MF had invaded the small intestine and could not be relieved intraoperatively. Finally, partial bowel resection, proximal stoma, and intravenous nutrition were performed to maintain life.
CONCLUSION Regular detection is the primary way to prevent deterioration from PJS. Although MF is a benign tumor, it has characteristics of invasive growth and ready recurrence. Therefore, close follow-up of both the history of MF and gastrointestinal surgery are advisable. Early detection and early treatment are the main means of improving patient prognosis.
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Affiliation(s)
- Huai-Jie Cai
- The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
| | - Han Wang
- The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
| | - Nan Cao
- The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
| | - Wei Wang
- The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
| | - Xi-Xi Sun
- The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
| | - Bin Huang
- Department of Ultrasound, Zhejiang Hospital, Hangzhou 310013, Zhejiang Province, China
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15
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Jin Z, Zhang Q, Tang D, Wang L, Wang W, Xu M, Li Z, Wang D. Successful treatment of giant mesenteric fibromatosis with surgery and tamoxifen: case report. Transl Cancer Res 2019; 8:996-1000. [PMID: 35116840 PMCID: PMC8798797 DOI: 10.21037/tcr.2019.05.13] [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: 12/12/2018] [Accepted: 04/26/2019] [Indexed: 02/05/2023]
Abstract
A 65-year-old man underwent excision of a giant mesenteric fibromatosis (MF) via combined splenectomy and partial transverse colectomy. Pathological examination confirmed the presence of MF, whereas genetic testing indicated that the tumor was sensitive to tamoxifen. Over a 1-year follow-up, no symptoms of abdominal discomfort or recurrence was noted.
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Affiliation(s)
| | - Qi Zhang
- Department of General Surgery, General Surgery Institute of Yangzhou, Northern Jiangsu Province Hospital, Clinical Medical College, Yangzhou University, Yangzhou 225001, China
| | - Dong Tang
- Department of General Surgery, General Surgery Institute of Yangzhou, Northern Jiangsu Province Hospital, Clinical Medical College, Yangzhou University, Yangzhou 225001, China
| | - Liuhua Wang
- Department of General Surgery, General Surgery Institute of Yangzhou, Northern Jiangsu Province Hospital, Clinical Medical College, Yangzhou University, Yangzhou 225001, China
| | - Wei Wang
- Department of General Surgery, General Surgery Institute of Yangzhou, Northern Jiangsu Province Hospital, Clinical Medical College, Yangzhou University, Yangzhou 225001, China
| | - Minghao Xu
- Dalian Medical university, Dalian 116044, China
| | - Zhi Li
- Dalian Medical university, Dalian 116044, China
| | - Daorong Wang
- Department of General Surgery, General Surgery Institute of Yangzhou, Northern Jiangsu Province Hospital, Clinical Medical College, Yangzhou University, Yangzhou 225001, China
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16
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Ballatori SE, Hecht JL, Lozano-Calderón SA. A Desmoid Tumor Involving the Subscapularis Muscle: A Case Report. JBJS Case Connect 2019; 9:e3. [PMID: 30628921 DOI: 10.2106/jbjs.cc.18.00184] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022]
Abstract
CASE A 31-year-old woman developed worsening pain and paresthesia in the neck, shoulder, and arm over a period of 6 years. Magnetic resonance imaging revealed a soft-tissue mass in the subscapular region, with likely involvement of the scapula and the subscapularis muscle. The mass was resected, and the final histologic diagnosis was desmoid-type fibromatosis. CONCLUSION Desmoid tumors with subscapularis muscle involvement are exceedingly rare. Although limited range of motion is the more common presentation for these tumors, this case demonstrates that desmoid tumors may present with primarily neurologic symptoms.
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Affiliation(s)
| | | | - Santiago A Lozano-Calderón
- Massachusetts General Hospital, Boston, Massachusetts.,Beth Israel Deaconess Medical Center, Boston, Massachusetts
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17
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Gondim Teixeira P, Chanson A, Verhaeghe JL, Lecocq S, Louis M, Hossu G, Blum A. Correlation between tumor growth and hormonal therapy with MR signal characteristics of desmoid-type fibromatosis: A preliminary study. Diagn Interv Imaging 2019; 100:47-55. [DOI: 10.1016/j.diii.2018.06.007] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2018] [Revised: 06/09/2018] [Accepted: 06/27/2018] [Indexed: 12/28/2022]
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Abstract
OBJECTIVE The purpose of this article is to review the etiopathogenesis, molecular cytogenetics, histopathology, clinical features, and multimodality imaging features of desmoid fibromatosis. Recent advances in the management of desmoid fibromatosis will also be discussed. CONCLUSIONS Desmoid fibromatosis is a rare soft tissue neoplasm with a high incidence of local recurrence. Imaging plays an important role in the diagnosis and management of this disease.
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Affiliation(s)
| | - Behrang Amini
- Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX
| | - Paul Nikolaidis
- Northwestern University, Feinberg School of Medicine, Chicago, IL
| | - Matthew Assing
- Department of Radiology, Stanford University School of Medicine, Stanford, CA
| | - Raghunandan Vikram
- Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX
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19
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Ning B, Jian N, Ma R. Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution. World J Surg Oncol 2018; 16:237. [PMID: 30563530 PMCID: PMC6299634 DOI: 10.1186/s12957-018-1536-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2018] [Accepted: 11/30/2018] [Indexed: 01/27/2023] Open
Abstract
Background and purpose Pediatric desmoid tumor (PDT) is rare and has a high local recurrence rate. The purpose of the present study was to analyze clinical risk factors of local recurrence in PDT patients. Materials and methods We reviewed clinical data of 66 PDT patients from 2004 to 2015. All patients underwent macroscopically complete resection, and some recurrent tumors were prescribed radiotherapy. Factors such as sex, age at presentation, location, and proximity to nerves or vasculature were analyzed. The local recurrence rate and recurrence-free survival were analyzed with these factors. Results All patients in the present study were children and had extra-abdominal tumors. The median follow-up time was 6.6 years. Thirty-six (55%) patients had local recurrence. Age, sex, tumor site, tumor size, and proximity to nerves/vasculature had a significant impact on prognosis in univariate analysis. Radiotherapy decreased the local recurrence rate. In multivariate analysis, younger age, tumor location in buttocks, larger tumor, and proximity to important nerves/vasculature were independent risk factors for poor prognosis. Conclusions Favorable therapeutic strategies could be selected according to the preoperative prognostic risk factors. Radiotherapy should be considered for local recurrence of PDT.
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Affiliation(s)
- Bo Ning
- Department of Pediatric Orthopaedic, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China
| | - Na Jian
- Department of Pediatric Orthopaedic, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China
| | - Ruixue Ma
- Department of Pediatric Orthopaedic, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China.
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20
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Seinen JM, Niebling MG, Bastiaannet E, Pras B, Hoekstra HJ. Four different treatment strategies in aggressive fibromatosis: A systematic review. Clin Transl Radiat Oncol 2018; 12:1-7. [PMID: 30069502 PMCID: PMC6067057 DOI: 10.1016/j.ctro.2018.03.001] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2018] [Revised: 02/20/2018] [Accepted: 03/02/2018] [Indexed: 12/26/2022] Open
Abstract
Relative good overall local control rate of all four different treatment groups of >75%. Multivariate analysis shows deep seated tumors, age (<30 years) and extracompartmentally situated tumors as negative predicting markers of local outcome. The radiotherapy alone group did not have better local control rates than the observation group (p = 0.355). In case of recurrent disease, adjuvant radiotherapy has a definite advantage over surgery alone (p = 0.001). Radiotherapy alone gives in 50% of cases partial or complete regression, and in 14% complete regression. Eventually 23% develop local progression. Observation could be considered as first line treatment in patients with tumors not in close adherence to important structures and no symptoms. Stabilization of the tumor arose after a median time of >1 year after observation, and a local recurrence or progression occurred after a median time of <3 years. Background The treatment approach for aggressive fibromatosis is changing. Although surgery is the mainstay in common practice, recent literature is reporting a more conservative approach. We compared the local control rate for surgery, surgery with radiotherapy, radiotherapy alone and a wait and see policy in a systematic review. Methods A comprehensive search of the databases PubMed/Medline, Embase and Cochrane, of the medical literature published in 1999 till March 2017 was performed by two reviewers, including articles about extra abdominal aggressive fibromatosis without the genetical variants. A total of 671 studies were assessed for eligibility, and 37 studies were included for analysis, representing 2780 patients. Results The local control rates for surgery alone, surgery and radiotherapy, radiotherapy alone and observation were 75%, 78%, 85% and 78%, respectively. For patients with recurrent disease observation had a better local control rate than surgery alone (p = 0.001). In the observation group, stabilization of the tumor was seen in median 14 (range 12–35) months. The time to local recurrence in the treatment group was median 17 (range, 11–52) months. Conclusion A watchful conservative first line approach with just observation and closely monitoring, by means of physical examination and MRI, appears to be justified in a subgroup of patients without clinical symptoms and no possible health hazards if the tumor would progress.
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Affiliation(s)
- Jojanneke M Seinen
- Department of Surgical Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands
| | - Maarten G Niebling
- Department of Surgical Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands
| | - Esther Bastiaannet
- Department of Surgery and Department of Gerontology & Geriatrics, Leiden University Medical Centre, The Netherlands
| | - Betty Pras
- Department of Radiation Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands
| | - Harald J Hoekstra
- Department of Surgical Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands
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21
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Tsagozis P, Stevenson JD, Grimer R, Carter S. Outcome of surgery for primary and recurrent desmoid-type fibromatosis. A retrospective case series of 174 patients. Ann Med Surg (Lond) 2017; 17:14-19. [PMID: 28386395 PMCID: PMC5374757 DOI: 10.1016/j.amsu.2017.03.023] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2016] [Revised: 03/20/2017] [Accepted: 03/20/2017] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The best management of relapsing desmoid-type fibromatosis, a benign but locally infiltrative soft-tissue tumour, is largely undecided. Our aim was to investigate the incidence and the factors influencing local relapse after surgery for primary and recurrent disease of the trunk and extremities. PATIENTS AND METHODS Retrospective analysis of 174 patients who had surgical treatment for desmoid-type fibromatosis. The quality of the surgical margins and use of adjuvant radiotherapy or chemotherapy were analysed regarding local recurrences in primary and recurrent disease. RESULTS Clear margins were achieved in 41% of cases. 10-year local control rate was 58% for clear primary resections as compared to 37% with intralesional primary resections (p = 0.030). Extremity tumours had a higher risk of local recurrence compared to trunk and pelvic ones (p < 0.001). Attempted resection of recurrent disease was associated with an approximately 90% incidence of relapse after each procedure, despite the quality of the surgical margins being equivalent to primary resections. Quality of surgical margins was not important for local control of recurrent lesions. Adjuvant treatments (radiotherapy and chemotherapy) had a no significant effect on the local control rate of recurrent disease (odds ratio 0.693 and 0.969 respectively). CONCLUSIONS A complete primary excision is the best window of opportunity to achieve local control of desmoid-type fibromatosis. Once the disease relapses, surgical intervention is accompanied with a high risk of failure, irrespective of the quality of the margins and adjuvant treatment given.
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Affiliation(s)
- Panagiotis Tsagozis
- The Royal Orthopaedic Hospital, Bristol Road South, Birmingham, B31 2AP, UK
- Section of Orthopaedics, Department of Molecular Medicine and Surgery, Karolinska University Hospital, Solna, 17176, Stockholm, Sweden
- Corresponding author. Present address: Section of Orthopaedics, Department of Molecular Medicine and Surgery, Karolinska University Hospital, Stockholm, Sweden.Section of OrthopaedicsDepartment of Molecular Medicine and SurgeryKarolinska University HospitalStockholmSweden
| | | | - Robert Grimer
- The Royal Orthopaedic Hospital, Bristol Road South, Birmingham, B31 2AP, UK
| | - Simon Carter
- The Royal Orthopaedic Hospital, Bristol Road South, Birmingham, B31 2AP, UK
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Janssen ML, van Broekhoven DLM, Cates JMM, Bramer WM, Nuyttens JJ, Gronchi A, Salas S, Bonvalot S, Grünhagen DJ, Verhoef C. Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg 2017; 104:347-357. [DOI: 10.1002/bjs.10477] [Citation(s) in RCA: 66] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2016] [Revised: 08/09/2016] [Accepted: 11/30/2016] [Indexed: 01/01/2023]
Abstract
Abstract
Background
Extra-abdominal desmoid-type fibromatosis (DF) is a rare, locally aggressive neoplasm that is usually managed conservatively. When treatment is indicated, it typically involves surgical resection, possibly with adjuvant radiotherapy. The indications for postoperative radiotherapy and its effectiveness are unclear. The objective of this study was to estimate the effect of surgical resection margins and adjuvant radiotherapy on rates of recurrence of DF.
Methods
Literature published between 1999 and 2015 was extracted from MEDLINE, Embase, Cochrane Central Registry of Trials, Web of Science and Google Scholar. Recurrence rate was analysed by meta-analysis and compared between subgroups.
Results
Sixteen reports were included, consisting of a total of 1295 patients with DF. In patients treated by surgical resection alone, the risk of local recurrence was almost twofold higher for those with microscopically positive resection margins (risk ratio (RR) 1·78, 95 per cent c.i. 1·40 to 2·26). Adjuvant radiotherapy after surgery with negative margins had no detectable benefit on recurrence. In contrast, after incomplete surgical resection, adjuvant radiotherapy improved recurrence rates both in patients with primary tumours (RR 1·54, 1·05 to 2·27) and in those with recurrent DF (RR 1·60, 1·12 to 2·28).
Conclusion
DF resected with microscopically positive margins has a higher risk of recurrence. Adjuvant radiotherapy appears to reduce the risk of recurrence after incomplete surgical resection, particularly in patients with recurrent tumours.
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Affiliation(s)
- M L Janssen
- Department of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands
| | - D L M van Broekhoven
- Department of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands
| | - J M M Cates
- Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Centre, Nashville, Tennessee, USA
| | - W M Bramer
- Department of Medical Library, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands
| | - J J Nuyttens
- Department of Radiation Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands
| | - A Gronchi
- Department of Soft Tissue/Bone Sarcoma and Melanoma, IRCCS Foundation National Cancer Institute, Milan, Italy
| | - S Salas
- Department of Medical Oncology and Palliative Care, University of Aix Marseille, Marseille, France
| | - S Bonvalot
- Department of Surgery, Curie Institute, Paris, France
| | - D J Grünhagen
- Department of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands
| | - C Verhoef
- Department of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands
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Li S, Fan Z, Fang Z, Liu J, Bai C, Xue R, Zhang L, Gao T. Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis. Chin J Cancer Res 2017; 29:455-462. [PMID: 29142465 DOI: 10.21147/j.issn.1000-9604.2017.05.10] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
Objective To assess the efficacy of conservative chemotherapy for inoperable desmoid tumor (DT) and analyze the prognostic factors. Methods From November 2008 to April 2016, 71 patients of inoperable DT were treated with vinorelbine and low-dose methotrexate in the Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, and enrolled in this retrospective study. The chemotherapy duration is one year. The efficacy of chemotherapy and the prognosis were observed. Results Of the 71 patients, 55% were female. Age of onset varied from 1 to 47 years, and the median age was 14 years. Only 11 (15.5%) cases suffered primary tumor. The distribution of the site of tumors was: 31 (43.7%) in the trunk, 36 (50.7%) in the limbs, and 4 (5.6%) in the peritoneal and pelvic cavity. The size of tumor (the maximum diameter) differed from 2 to 37 cm with a mean of 9.3 cm. The median follow-up duration was 28 (range, 6-87) months. Common side effects included: nausea and vomiting, liver injury, bone marrow suppression and oral ulcers. When the chemotherapy finished, 1 (1.4%) case achieved complete response, 24 (33.8%) achieved partial response, 37 (52.1%) achieved stable disease and 9 (12.7%) had progressive disease. The overall response rate was 87.3%. The progression-free survival (PFS) of the participants were from 6 to 87 months, and the 2-, 3- and 5-year PFS was 79.9%, 68.4% and 36.3%, respectively. No significant difference was identified in PFS in subgroups of gender, age of onset, age of chemotherapy, tumor site and tumor size. Conclusions For recurrent, inoperable and progressive DT, enough course of chemotherapy with vinorelbine combined with low-dose methotrexate was an optional choice for local control.
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Affiliation(s)
- Shu Li
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Zhengfu Fan
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Zhiwei Fang
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Jiayong Liu
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Chujie Bai
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Ruifeng Xue
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Lu Zhang
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
| | - Tian Gao
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, Beijing 100142, China
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Quast DR, Schneider R, Burdzik E, Hoppe S, Möslein G. Long-term outcome of sporadic and FAP-associated desmoid tumors treated with high-dose selective estrogen receptor modulators and sulindac: a single-center long-term observational study in 134 patients. Fam Cancer 2016; 15:31-40. [PMID: 26275868 DOI: 10.1007/s10689-015-9830-z] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Aim of this study is to evaluate the outcome of long-term conservative treatment with sulindac and high-dose selective estrogen receptor modulators (SERMs) for sporadic and FAP-associated desmoid tumors. Desmoids are very rare tumors in the general population but occur frequently in FAP patients, being encountered in 23-38 %. Treatment of desmoids is still most controversial since response cannot be predicted and they are prone to develop recurrence. This study included all desmoid patients that were treated and followed at our institution and had completed at least 1 year of treatment. Response was defined as stable size or regression of desmoid size between two CT or MRI scans. A total of 134 patients were included. 64 (47.8 %) patients had a confirmed diagnosis of FAP, 69 (51.5 %) patients were sporadic. Overall 114 (85.1 %) patients showed regressive or stable desmoid size. Patients with previous history of multiple desmoid-related surgeries showed less-favorable response. The mean time to reach at least stable size was 14.9 (±9.1) months. After regression or stabilization, medication was tapered in 69 (60.5 %) of the treated patients with only one long-term recurrence after >10 years. The results of this study fortify the role of sulindac and high-dose SERMs as an effective and safe treatment for both, sporadic and FAP-associated desmoid tumors. While invasive treatment frequently results in high recurrence rates, high morbidity and high mortality, this conservative treatment is successful in most patients. The recurrence rate is negligible with no desmoid-related mortality in this large series. Therefore surgical resection, especially for mesenteric desmoids, should be deferred favoring this convincingly effective, well tolerated regimen.
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Affiliation(s)
- Daniel Robert Quast
- Klinik für Allgemeine Innere Medizin - Medizinische Klinik I, St. Josef-Hospital, Gudrunstr. 56, 44791, Bochum, Germany.
| | - Ralph Schneider
- Klinik für Hereditäre Tumorerkrankungen, HELIOS-Klinikum Wuppertal, Heusnerstr. 40, 42283, Wuppertal, Germany
| | - Emanuel Burdzik
- Klinik für Allgemein- und Viszeralchirurgie, Evangelisches Krankenhaus Bethesda zu Duisburg, Heerstraße 219, 47053, Duisburg, Germany
| | - Steffen Hoppe
- Klinik für Radiologie, HELIOS St. Josefs-Hospital Bochum-Linden, Axstr. 35, 44879, Bochum, Germany
| | - Gabriela Möslein
- Klinik für Hereditäre Tumorerkrankungen, HELIOS-Klinikum Wuppertal, Heusnerstr. 40, 42283, Wuppertal, Germany.
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26
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Park JS, Nakache YP, Katz J, Boutin RD, Steffner RJ, Monjazeb AM, Canter RJ. Conservative management of desmoid tumors is safe and effective. J Surg Res 2016; 205:115-20. [DOI: 10.1016/j.jss.2016.06.028] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2016] [Revised: 05/11/2016] [Accepted: 06/09/2016] [Indexed: 12/15/2022]
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Mueller C, Croner R, Klein P, Grützmann R, Vassos N. Primary and recurrent sporadic desmoids: Prognostic factors influencing recurrence-free survival after complete gross resection. Int J Surg 2016; 31:63-70. [PMID: 27262879 DOI: 10.1016/j.ijsu.2016.05.068] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2016] [Revised: 05/18/2016] [Accepted: 05/26/2016] [Indexed: 10/21/2022]
Abstract
INTRODUCTION There is continuing controversy over the significance of prognostic factors in sporadic desmoid-type fibromatosis (DF). Further, only limited data is available in the literature concerning second recurrence in patients with recurrent DF. The purpose of this study was to identify prognostic factors influencing recurrence-free survival (RFS) and to determine rates of local recurrence (LR) in patients with primary and recurrent DF. METHODS Fifty-three consecutive patients with DF who underwent surgery at a single institution were identified. As a result of strict exclusion criteria, a homogeneous cohort of thirty-two patients who received macroscopically complete resection was included in this study and split into a primary (PG) and recurrent disease (RG) group. Clinicopathological parameters and immunohistochemical markers were retrospectively and independently analyzed in both groups. RESULTS The local recurrence rate was 35% for the PG and 33% for the RG. The PG's calculated median time to relapse was 17 months, and the RG's was 29 months. The estimated 1-, 5-, and 8-year RFS rates for the PG were 75%, 64%, and 55% while the corresponding RFS rates for the RG were 69%, 69%, and 52%, respectively. In primary disease, extraabdominal tumor location was the only significant adverse prognostic factor associated with worse RFS (p = 0.008). Whereas in recurrent disease, age ≥40 yrs (p = 0.022) and R0 margin status (p = 0.049) correlated with a significantly better outcome. CONCLUSION Recurrence in primary and recurrent DF is characterized by different predictive factors and benefits from distinct therapeutic strategies. Immunohistochemical markers are valuable tools in the diagnosis of DF, although have limited potential in predicting the outcome.
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Affiliation(s)
- Christian Mueller
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany; Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany
| | - Roland Croner
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | - Peter Klein
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | - Robert Grützmann
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | - Nikolaos Vassos
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany.
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28
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Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagare AB, Hornick JL, Baldini EH, George S, Ramaiya NH, Jagannathan JP. Role of Imaging in Management of Desmoid-type Fibromatosis: A Primer for Radiologists. Radiographics 2016; 36:767-82. [DOI: 10.1148/rg.2016150153] [Citation(s) in RCA: 76] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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29
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Burtenshaw SM, Cannell AJ, McAlister ED, Siddique S, Kandel R, Blackstein ME, Swallow CJ, Gladdy RA. Toward Observation as First-line Management in Abdominal Desmoid Tumors. Ann Surg Oncol 2016; 23:2212-9. [DOI: 10.1245/s10434-016-5159-6] [Citation(s) in RCA: 50] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2015] [Indexed: 11/18/2022]
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Meshikhes AW, Al-Zahrani H, Ewies T. Laparoscopic excision of abdominal wall desmoid tumor. Asian J Endosc Surg 2016; 9:79-82. [PMID: 26781534 DOI: 10.1111/ases.12257] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/10/2015] [Revised: 09/30/2015] [Accepted: 10/12/2015] [Indexed: 02/03/2023]
Abstract
Open surgical resection is the mainstay treatment for desmoid tumors. Laparoscopic resection is rarely used and not well described in the literature. We report a case of a single, 35-year-old woman who presented with palpable abdominal wall desmoid tumor. The patient had had laparoscopic cholecystectomy 2 years earlier, and the tumor was at the insertion site of the right upper quadrant trocar. The diagnosis was made by a Tru-Cut biopsy at another institution, after the lesion had increased in size and caused increased discomfort. The patient underwent successful laparoscopic resection of the tumor. This report aimed to promote laparoscopic resection of abdominal wall desmoid tumors, whenever feasible, and describe the laparoscopic technique. We believe this is the second case of laparoscopic excision of desmoid tumor reported in the English-language literature.
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Affiliation(s)
- Abdul-Wahed Meshikhes
- Section of General and Minimally Invasive Surgery, Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Hana Al-Zahrani
- Section of General and Minimally Invasive Surgery, Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Tarek Ewies
- Section of General and Minimally Invasive Surgery, Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
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31
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Al-Jazrawe M, Au M, Alman B. Optimal therapy for desmoid tumors: current options and challenges for the future. Expert Rev Anticancer Ther 2015; 15:1443-58. [DOI: 10.1586/14737140.2015.1096203] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
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Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: Analysis of 114 patients at a single institution. Eur J Surg Oncol 2015; 41:1013-9. [PMID: 26005133 DOI: 10.1016/j.ejso.2015.04.016] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2015] [Revised: 03/23/2015] [Accepted: 04/12/2015] [Indexed: 12/19/2022] Open
Abstract
AIMS Desmoid-type fibromatoses (DFs) are rare soft-tissue neoplasms with frequent local recurrence. We sought to determine the prognostic factors that are predictive of recurrence-free survival (RFS) for these tumors. METHODS One hundred and fourteen consecutive patients with sporadic DF who received macroscopically complete resection (R0/R1) at a single tertiary hospital between 1985 and 2014 were included. A total of 10 clinical and pathological parameters were analyzed. Histologic slides and the margin status were re-checked; close margins (≤1-mm clearance) were noted separately and were considered together with the R1 margin. RESULTS The median follow-up interval was 72.5 months. Thirty-five (30.7%) patients had a local recurrence. The 2-, 5- and 10-year RFSs were 75.2%, 72.1% and 67.0%, respectively. In univariate analysis, age, tumor size, tumor site, margin status and presence of lesions at multiple sites had a significant impact on RFS. In multiple analysis, younger age (age<30 vs. age≥50 years: hazard ratio [HR] = 4.96; 95% confidence interval [95% CI], 1.50-16.4; p = 0.009); an extra-abdominal site (extra-abdominal site vs. other sites: HR = 4.08; 95% CI, 1.49-11.2; p = 0.006); larger tumor size (≥8 cm vs. <8 cm: HR = 2.43; 95% CI, 1.15-5.13; p = 0.021); and close or positive margin status (close margin/R1 vs. R0: HR = 2.64; 95% CI, 1.11-6.25; p = 0.027) were independent, unfavorable prognostic factors. CONCLUSIONS Different prognostic subgroups were identified that allow for the better selection of favorable therapeutic strategies. The role of the margin status should be considered with caution and should be based on a more precise pathological result.
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Efthimiopoulos GA, Chatzifotiou D, Drogouti M, Zafiriou G. Primary asymptomatic desmoid tumor of the mesentery. AMERICAN JOURNAL OF CASE REPORTS 2015; 16:160-3. [PMID: 25782060 PMCID: PMC4371711 DOI: 10.12659/ajcr.892521] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Patient: Male, 40 Final Diagnosis: Mesenteric desmoid tumor Symptoms: Abdominal mass Medication: — Clinical Procedure: Laparotomy exploratory Specialty: Surgery
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Affiliation(s)
- George Ap Efthimiopoulos
- 1st Department of Surgical Oncology, "Theagenio" Cancer Hospital of Thessaloniki, Thessaloniki, Greece
| | - Dimitrios Chatzifotiou
- 1st Department of Surgical Oncology, "Theagenio" Cancer Hospital of Thessaloniki, Thessaloniki, Greece
| | - Maria Drogouti
- 1st Department of Surgical Oncology, "Theagenio" Cancer Hospital of Thessaloniki, Thessaloniki, Greece
| | - George Zafiriou
- 1st Department of Surgical Oncology, "Theagenio" Cancer Hospital of Thessaloniki, Thessaloniki, Greece
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Large desmoid-type fibromatosis of the shoulder girdle: operative approach selection and clinic outcome. INTERNATIONAL ORTHOPAEDICS 2015; 39:363-9. [PMID: 25662763 DOI: 10.1007/s00264-014-2660-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Accepted: 12/24/2014] [Indexed: 02/05/2023]
Abstract
PURPOSE Surgical resection of large desmoid-type fibromatosis (DTF) of the shoulder girdle is challenging. This article discusses the operative approach and evaluates the clinic outcome. METHOD Twenty-one cases between June 2003 and June 2012 were retrospectively reviewed. Eleven patients newly diagnosed and ten had previous operations. Mean tumour size was 10.5 cm (range, 7.0-19.0 cm). We divided the shoulder girdle into four regions; the adopted surgical resection was en bloc in 18 cases and piecemeal resection in three. In nine cases we took an anterior approach, ten a posterior approach and two a combined anteroposterior (AP) approach. Brachytherapy with permanent I(125) implantation was applied in all cases. RESULT All 21 patients were followed up for a mean of 49.8 (range, 18-136) months. Recurrence was seen in two patients, with the overall recurrence rate of 9.5 %. The Musculoskeletal Tumour Score (MSTS) was between 56.7 and 96.7 %, with the average at 78.9 % and excellent-good rate 95.2 %. Complications were found in three of the 21 patients (14.3 %). CONCLUSION Surgical resection with the precondition of preserving vital structure for large DTF of the shoulder girdle is necessary. According to our new partition, it was suitable to take an anterior approach when regions I or I + II were involved, posterior approach for regions II, III or II + III; combined AP approach when involving region I + II + III; and anterior or posterior approach for region IV, depending upon tumour location.
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