Article

Successful Resection of Locally Advanced Gastrointestinal Stromal Tumor of the Ampulla of Vater after Treatment with Imatinib

Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Kyung Hee University, Seoul, Korea.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi 07/2010; 56(1):39-44. DOI: 10.4166/kjg.2010.56.1.39
Source: PubMed

ABSTRACT

Gastrointestinal stromal tumor (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract arising from Cajal's cells, expressing CD 117. The standard treatment for primary GIST is complete surgical resection. Imatinib mesylate, a specific tyrosine kinase inhibitor, is effective against locally advanced and metastatic GIST. There are several reports of the effect of preoperative imatinib in patients with unresectable and locally advanced primary GIST. We report a case of unresectable primary GIST of the ampulla of Vater, which we were able to completely resect after treatment with a dosage of imatinib 400 mg daily for 5 months. Twelve months later, the patient was treated with imatinib and doing well with no evidence of recurrence.

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    ABSTRACT: The article presents a clinical case of the gastrointestinal stromal tumor of the duodenum. In a 74-year-old woman during the surgery for stenosis of the Vater's papilla and obstructive jaundice, the tumor in the 3 section of the duodenum, 4 cm in diameter was revealed. Cholecystectomy, wedge resection of the duodenal tumor, transduodenal papillosphincterotomy and papillosphincteroplasty with the duodenal defect suturing and choledochostomy on Halsted were carried out in the patient. At the pathohistological study, the removed formation was presented as a gastrointestinal stromal tumors (GIST) of the duodenum (CD 117+). The patient hadn't any recurrence during 6 months after the surgery. The data concerning epidemiology, clinical manifestation, diagnostics and treatment methods of this pathology are presented on the basis of the literature review. Duodenal GIST is a rare disease, characterized by a predominantly local expansion, rarely involving lymph nodes and no tendency to metastases. Resection within healthy tissue and segmental resection of the duodenum are the method of choice at the treatment of duodenal GIST. Surgical treatment of extensive primary tumors or the metastasis or recurrent GIST of the duodenum should be integrated into a multimodal therapeutic concept that includes pancreatoduodenal resection and the targeted therapy with tyrosine kinase inhibitors.
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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) usually develop in the stomach and small intestine and only rarely occur at the ampulla of Vater, with only 11 cases reported in the literature. We report a case of a GIST of the ampulla of Vater. A 36-year-old, previously healthy man presented with a loss of consciousness lasting a few minutes. A gastroduodenal endoscopy revealed a submucosal tumor with central ulceration at the ampulla of Vater. The enhanced computed tomography scan revealed a smooth-outlined hypervascular solid mass (24 mm × 30 mm) in the second part of the duodenum. Neither lymphadenopathy nor metastasis was observed. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed normal bile and pancreatic ducts. Biopsies were collected from the ulcerative lesion, and the tumor was diagnosed as a GIST. A submucosal tumor with central ulceration may be a characteristic form of GISTs of the ampulla of Vater, and biopsy studies are useful for the diagnosing such tumors. The patient underwent pancreatoduodenectomy, and the operative specimen revealed a 2.2-cm GIST with 1 mitosis per 50 high-power fields. The gold standard for treatment of GISTs is surgical resection without rupture of a capsule. If technically possible, local resection may be considered. However, when the location of the lesion presents challenges, a pancreatoduodenectomy should be performed for GIST of the ampulla of Vater.
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