Gastric Desmoid Tumor: An Infrequent Case of Intra-Abdominal Fibromatosis
Gastric Esophageal Pathology Unit, Department of General Surgery and Gastroenterology, University Welfare Complex of Salamanca, Salamanca, Castilla y Leon, Spain
- *Corresponding Author:
- Dr. Omar Abdel-lah Fernández
Gastric Esophageal Pathology Unit
Department of General Surgery and Gastroenterology
University Welfare Complex of Salamanca
Salamanca, Castilla y Leon, Spain
Tel: +34 923261198
E-mail: [email protected]
Received date: August 10, 2015 Accepted date: September 02, 2015 Published date: September 09, 2015
Citation: Macias N, Abdel-lah O, Parreño FC, Blanco O, Bengoechea O, et al. (2015) Gastric Desmoid Tumor: An Infrequent Case of Intra-Abdominal Fibromatosis. J Gastrointest Dig Syst 5:332. doi:10.4172/2161-069X.1000332
Copyright: © 2015 Macias N, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Background and purpose: Desmoid tumors or aggressive fibromatosis are infrequent conditions, with a large clinical variability, and preferencial location on abdominal wall, extra-abdominal soft tissue, and mesentery. Histologically benign but locally aggressive, they have a marked tendency to recurrence. There are two known variants: sporadic and associated to familial adenomatous polyposis. Its etiology remains unknown, but it appears to be related to estrogenic estimulation, surgical aggression and mutations of the short arm of chromosome 5. Diagnosis is usually difficult, and must combine medical history, semiology and imaging, though only histological analysis of the specimen will provide a definitive diagnosis after surgical resection, which is potentially curative. Gastric location has not been reported so far. Case report: 37-year-old woman with recent delivery, presenting abdominal lump with non specific clinical semiology and rapid growth rate. After diagnostic tests, a hypervascular mass of about 15 centimeter of diameter, depending on gastric wall is found. She underwent an elective distal gastrectomy and Billroth I reconstruction. Histology confirms a mesenchymal desmoid gastric tumor. Discussion: The differential diagnosis for abdominal oligosymptomatic lumps which respect the mucosa of the gastrointestinal tract lead clinical suspicion to mesenchymal tumors such as sarcomas, desmoids, or GIST. Radiologic tests are useful to confirm resectability and detect complications. When facing unresectable disease, planning a biopsy and systemic treatment with chemotherapy and/or radical radiotherapy should be considered. Diagnosis is reached after histological and inmunohistochemical analysis of the specimen, which in case of a desmoid, will show negative expression to markers of sarcoma (actin, desmin, S100) or GIST (CD117, DOG1, PDGFRA), and positive staining with anti-beta-catenin. Conclusion: Desmoid tumors should be considered in the differential diagnosis of abdominal oligosymptomatic masses, specially in fertile women or if history of surgical trauma. Patients with desmoid tumors should undergo colonic polyposis screening, as well as patients with adenomatous polyposis and an abdominal lump should lead suspected diagnosis to the possibility of a desmoid tumor.