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Kim JH, Ryu MH, Park YS, Kim HJ, Park H, Kang YK. Intra-abdominal desmoid tumors mimicking gastrointestinal stromal tumors - 8 cases: A case report. World J Gastroenterol 2019; 25:2010-2018. [PMID: 31086468 PMCID: PMC6487383 DOI: 10.3748/wjg.v25.i16.2010] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2018] [Revised: 02/22/2019] [Accepted: 03/01/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Intra-abdominal desmoid tumors (DTs) can mimic recurrence or progression of gastrointestinal stromal tumors (GISTs). Differential diagnosis is important to avoid unnecessary or inappropriate treatment. CASE SUMMARY All 8 patients experienced surgical resection of GIST, and median time to diagnosis of DT was 1.8 years after surgical resection. All sites of DT were in the peritoneum around the surgical sites of GIST. The following clinical suspicion coupled with radiological findings contributed to the suspicion of intra-abdominal DTs: (1) Occurrence of a new single lesion in the peritoneum around the surgical sites of GIST; (2) uncontrolled lesion with imatinib while other lesions being controlled with imatinib; (3) well-defined ovoid shaped lesion with delayed or mild enhancement and absence of necrosis, hemorrhage, and cystic change on computed tomography; and (4) a lesion showing mild or no hypermetabolic activity on 18fluorodeoxyglucose-positron emission tomography, contrary to initially hyperactive lesion of GIST. All DTs were surgically removed except for one unresectable DT and only one DT recurred at another site of peritoneum, which was also surgically removed. CONCLUSION Intra-abdominal DT should be a differential diagnosis for a new single lesion in patients with GIST.
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Affiliation(s)
- Jwa Hoon Kim
- Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
| | - Min-Hee Ryu
- Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
| | - Young Soo Park
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
| | - Hyun Jin Kim
- Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
| | - Hyojung Park
- Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
| | - Yoon-Koo Kang
- Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, South Korea
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Wang Z, Wu J, Lv A, Tian X, Hao C. En bloc resection for intra-abdominal/retroperitoneal desmoid-type fibromatosis with adjacent organ involvement: A case series and literature review. Biosci Trends 2019; 12:620-626. [PMID: 30674762 DOI: 10.5582/bst.2018.01285] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Surgical treatment for intra-abdominal/retroperitoneal desmoid-type fibromatosis (IA/RPDF) is still controversial. Studies regarding en bloc resection in IA/RPDF with adjacent organ involvement are scanty. This study aims to evaluate the safety and effectiveness of en bloc resection in IA/RPDF with adjacent organ involvement. This retrospective clinical study included 21 patients who were diagnosed with IA/RPDF and underwent tumor resection at a single center between March 2013 and June 2018. All patients included in the study underwent surgery with curative intent, and IA/RPDF with adhesive organs was removed en bloc. The safety of surgical treatment was verified by the analysis of intraoperative bleeding, postoperative morbidity and perioperative mortality. The efficacy of surgical treatment was evaluated based on the status of tumor infiltration of adjacent organs and patient follow-up results. Complete macroscopic (R0 or R1) resection was achieved in all cases. A median of 2 (range, 1-7) organs were resected. The median operating time was 300 (90-650) minutes. The median intraoperative bleeding was 300 (20-4,500) milliliters. For postoperative pathological diagnosis at our center, tumor infiltrated at least one organ in each patient. Infiltration was noted in 45 resected organs (45/57, 78.9%). Grade III-V postoperative morbidity developed in one patient (4.8%). During the follow-up, one patient developed local recurrence. No DF-related death was noted during the follow-up. The 3-year disease-free survival rate was 94.1% (95% confidence interval: 83.6-100%). Therefore, en bloc resection of the tumor and involved adjacent organs is a safe and effective treatment modality for IA/RPDF.
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Affiliation(s)
- Zhen Wang
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Hepato-PancreatoBiliary Surgery, Peking University Cancer Hospital and Institute
| | - Jianhui Wu
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Hepato-PancreatoBiliary Surgery, Peking University Cancer Hospital and Institute
| | - Ang Lv
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Hepato-PancreatoBiliary Surgery, Peking University Cancer Hospital and Institute
| | - Xiuyun Tian
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Hepato-PancreatoBiliary Surgery, Peking University Cancer Hospital and Institute
| | - Chunyi Hao
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Hepato-PancreatoBiliary Surgery, Peking University Cancer Hospital and Institute
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Subsequent Development of Desmoid Tumor after a Resected Gastrointestinal Stromal Tumor. Case Rep Pathol 2018; 2018:1082956. [PMID: 29854525 PMCID: PMC5954958 DOI: 10.1155/2018/1082956] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Accepted: 03/26/2018] [Indexed: 11/17/2022] Open
Abstract
Desmoid tumors (deep fibromatosis) of the mesentery are rare mesenchymal tumors. They are often misdiagnosed, especially with a previous history of resection for gastrointestinal stromal tumor (GIST). Immunohistochemistry can help differentiate between these two tumors. In this article, we present a case we had encountered: a Desmoid tumor developing in a patient with a history of GIST 3 years ago. It is the first case of GIST with subsequent development of Desmoid tumor to be reported in Saudi Arabia. We discuss the two entities of Desmoid tumor and GIST by comparing their definitions, clinical presentations, histological features, immunohistochemistry stains, molecular pathogenesis, prognosis, and treatment. We also discuss the relationship between GIST and the subsequent development of Desmoid tumors and compare our case with case reports in literature.
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Thway K, Abou Sherif S, Riddell AM, Mudan S. Fibromatosis of the Sigmoid Colon With CTNNB1 (β-Catenin) Gene Mutation, Arising at the Site of Ileocolic Anastomosis for Resection of Gastrointestinal Stromal Tumor. Int J Surg Pathol 2016; 24:264-8. [PMID: 26721303 DOI: 10.1177/1066896915620012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
We describe a case of intra-abdominal fibromatosis, which occurred in a 44-year-old woman who had a previous history of gastrointestinal stromal tumor (GIST) of the sigmoid mesocolon, which was treated with imatinib and resection. A mass was detected at the site of ileocolic anastomosis of the previous small bowel resection and sigmoid colectomy, nearly 3 years later. Clinically, this was suspected to represent recurrent GIST and was excised, but histology and mutational analysis showed desmoid-type fibromatosis with a mutation in codon 41 of exon 3 of the CTNNB1 (β-catenin) gene. The occurrence of fibromatosis at the site of excision of GIST is very rare, but its recognition is important as the treatment of the two neoplasms differs significantly. As imaging cannot reliably distinguish between these 2 entities, histological diagnosis is crucial for correct clinical management.
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Jiang D, He D, Hou Y, Lu W, Shi Y, Hu Q, Lu S, Xu C, Liu Y, Liu J, Tan Y, Zhu X. Subsequent intra-abdominal fibromatosis mimicking recurrent gastrointestinal stromal tumor. Diagn Pathol 2013; 8:125. [PMID: 23902675 PMCID: PMC3751073 DOI: 10.1186/1746-1596-8-125] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2013] [Accepted: 07/04/2013] [Indexed: 02/06/2023] Open
Abstract
Abstract Intra-abdominal fibromatosis (IAF) commonly develops in patients who had abdominal surgery. In rare instances, it occurs subsequent to gastrointestinal stromal tumor (GIST). This special situation has clinical significance in imatinib era. About 1000 patients with GIST in our institution from 1993 to 2010 were re-evaluated based on their clinical and pathological data, the treatment strategies and the follow-up information. We identified 2 patients who developed IAF after GIST resection. Patient 1 was a 54 year-old male and had 5 cm × 4.5 cm × 3.5 cm jejunal GIST excised on February 22, 1994. Three years later, an abdominal mass with 7 cm × 6 cm × 3 cm was identified. He was diagnosed as recurrent GIST from clinical point of view. After excision, the second tumor was confirmed to be IAF. Patient 2 was a 45-year-old male and had 6 cm × 4 cm × 3 cm duodenal GIST excised on August 19, 2008. One year later, a 4 cm mass was found at the original surgical site. The patient refused to take imatinib until the tumor increased to 8 cm six months later. The tumor continued to increase after 6 months’ imatinib therapy, decision of surgical resection was made by multidisciplinary team. The second tumor was confirmed to be IAF with size of 17 cm × 13 cm × 11 cm. Although IAF subsequent to GIST is very rare, it is of clinical significance in imatinib era as an influencing factor for making clinical decision. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1076715989961803
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Affiliation(s)
- Dongxian Jiang
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, PR China
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Shih LY, Wei CK, Lin CW, Tseng CE. Postoperative retroperitoneal desmoid tumor mimics recurrent gastrointestinal stromal tumor: A case report. World J Gastroenterol 2012; 18:6172-6. [PMID: 23155350 PMCID: PMC3496898 DOI: 10.3748/wjg.v18.i42.6172] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2012] [Revised: 07/23/2012] [Accepted: 08/14/2012] [Indexed: 02/06/2023] Open
Abstract
Desmoid tumor is a locally invasive, myofibroblastic, nonmetastatic tumor. Its pathogenesis remains unclear and it may involve genetic abnormalities, sex hormones and traumatic injury, including surgery. Postoperative intra-abdominal desmoid tumor is rare, especially in the retroperitoneum. We report a case of postoperative retroperitoneal desmoid tumor that developed 29 mo after the first excision of a gastrointestinal stromal tumor. Sporadic trauma-related intra-abdominal desmoid tumors reported in the English literature are also reviewed. Despite an extremely low incidence, postoperative desmoid tumor should be considered in the differential diagnosis when a recurrent neoplasm is found at least one year after operation. However, it is a clinical challenge to distinguish recurrent malignant neoplasms from desmoid tumors, and surgical resection is the treatment option depending on the anatomic location.
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Abstract
Desmoid tumors are rare neoplasms. They occur mostly in females in their reproductive age and may present with pelvic pain and intestinal obstruction. These connective tissue neoplasms are because of uncontrolled proliferation of differentiated myofibroblasts. The cells may synthesize vast amounts of collagen fibers in response to various stimuli. We describe a case of a pelvic desmoid tumor simulating a uterine leiomyoma recurrence. We review the literature on the epidemiology and the treatment options for desmoid tumors and suggest a strong index of suspicion when a new pelvic mass arises in an adult with previous pelvic surgery. We advise a planned multidisciplinary treatment approach at the first hint of the diagnosis of desmoid tumor.
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Dumont AG, Rink L, Godwin AK, Miettinen M, Joensuu H, Strosberg JR, Gronchi A, Corless CL, Goldstein D, Rubin BP, Maki RG, Lazar AJ, Lev D, Trent JC, von Mehren M. A nonrandom association of gastrointestinal stromal tumor (GIST) and desmoid tumor (deep fibromatosis): case series of 28 patients. Ann Oncol 2012; 23:1335-1340. [PMID: 21994214 PMCID: PMC3493136 DOI: 10.1093/annonc/mdr442] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2011] [Revised: 08/12/2011] [Accepted: 08/17/2011] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND Gastrointestinal stromal tumors (GISTs) and desmoid tumors (DTs) are two rare mesenchymal tumor. Anecdotal reports of individuals with both diseases led us to make the hypothesis that the association is a nonrandom event as the probability would be extremely low to observe such cases if they were independent events. PATIENTS AND METHODS We evaluated the existence of patients with GIST and DT in a large multicenter cohort at 10 institutions in the United States, Australia and Europe. Data on gender, age at diagnosis, KIT, PDGFRA, CTNNB1 mutation status and follow-up time after diagnosis were collected. RESULTS We identified 28 patients diagnosed with both tumors. DT was diagnosed after GIST in 75% of patients and concomitantly in 21%. In only one case (4%), GIST was diagnosed after DT. KIT or PDGFRA mutations were detected in 12 of 14 GIST, 9 in KIT exon 11, 2 in KIT exon 9 and 1 in PDGFRA. CONCLUSION A statistical analysis of these 28 cases suggests a nonrandom association between GIST and DT. Further studies may be able to elucidate the underlying biology responsible for this association.
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Affiliation(s)
- A G Dumont
- Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston
| | - L Rink
- Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia
| | - A K Godwin
- Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia; Department of Pathology and Laboratory Medicine, University of Kansas Medical Center
| | - M Miettinen
- Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, USA
| | - H Joensuu
- Department of Oncology, Helsinki University Central Hospital, Helsinki, Finland
| | - J R Strosberg
- Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, USA
| | - A Gronchi
- Department of Surgery, National Tumor Institute, Milan, Italy
| | - C L Corless
- Department of Pathology and Knight Cancer Institute, Oregon Health & Science University, Portland, USA
| | - D Goldstein
- Department of Medical Oncology, Prince of Wales Hospital, Sydney, Australia
| | - B P Rubin
- Department of Molecular Genetics, Lerner Research Institute; Department of Anatomic Pathology, Taussig Cancer Center, Cleveland Clinic, Cleveland
| | - R G Maki
- Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York
| | - A J Lazar
- Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston
| | - D Lev
- Department of Cancer Biology, The University of Texas M. D. Anderson Cancer Center, Houston, USA
| | - J C Trent
- Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston.
| | - M von Mehren
- Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia
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