Greater omentum gastrointestinal stromal tumor withPDGFRA-mutation and hemoperitoneum
Open Access
- 1 January 2012
- journal article
- Published by Baishideng Publishing Group Inc. in World Journal of Gastrointestinal Oncology
- Vol. 4 (5), 119-124
- https://doi.org/10.4251/wjgo.v4.i5.119
Abstract
Although gastrointestinal stromal tumor (GIST) occurs generally in the digestive tract, omental GIST is very rare. We report the first case of an adult greater omental GIST with a new platelet-derived growth factor receptor α gene (PDGFRA)-mutation with hemoperitoneum. A 43-year-old man was admitted to our hospital complaining of acute abdominal pain. Abdominal contrast-enhanced computed tomography revealed a huge mass in the right abdominal cavity, and a large accumulation of fluid in the pelvic cavity, suggesting hemoperitoneum. We diagnosed the rupture as an intra-abdominal tumor, and an emergency tumorectomy was performed with resection of the greater omentum. This tumor was located in the distal right side of the greater omentum, and showed no continuity with the gastric wall. The tumor occurred primarily in the greater omentum. The resected tumor was about 19 cm × 12 cm × 14 cm in diameter, and weighed 1529 g. Histologically, the tumor was composed of epithelioid-shaped cells with high cellularity, and was positive for CD117 and CD34, and negative for S-100, α-smooth muscle actin. The mitosis was 6/50 under high power field. This case showed exon 18 mutation of PDGFRA with 846 (Asp to Glu) substitution, 848 (Asn to Lys) substitution. This is the first report of this PDGFRA mutation in omental GIST, and this might play an important role in the tumorigenesis of this case. Based on these findings, the tumor was diagnosed as high risk GIST primarily occurring in the greater omentum. The patient was treated with imatinib at a dose of 400 mg/d as adjuvant chemotherapy, and has been followed up for 24 mo with no evidence of recurrence.Keywords
This publication has 27 references indexed in Scilit:
- Gastrointestinal Stromal Tumors Presenting as Omental Masses—A Clinicopathologic Analysis of 95 CasesThe American Journal of Surgical Pathology, 2009
- A malignant omental extra-gastrointestinal stromal tumor on a young man: a case report and review of the literatureWorld Journal of Surgical Oncology, 2008
- Primary omental Gastrointestinal stromal tumor (GIST)World Journal of Surgical Oncology, 2007
- KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumoursEuropean Journal of Cancer, 2006
- PDGFRA Mutations in Gastrointestinal Stromal Tumors: Frequency, Spectrum and In Vitro Sensitivity to ImatinibJournal of Clinical Oncology, 2005
- Pediatric gastrointestinal stromal tumors and leiomyosarcomaCancer, 2004
- Prognostic Significance of c-kit Mutation in Localized Gastrointestinal Stromal TumorsClinical Cancer Research, 2004
- Kinase Mutations and Imatinib Response in Patients With Metastatic Gastrointestinal Stromal TumorJournal of Clinical Oncology, 2003
- Gain-of-function mutations of platelet-derived growth factor receptor α gene in gastrointestinal stromal tumorsGastroenterology, 2003
- Gain-of-Function Mutations of c- kit in Human Gastrointestinal Stromal TumorsScience, 1998