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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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 | Fig. 1.Abdominal CT scan findings. (A) Enhanced CT scan showed a 7.6×5.3 cm sized mass in the duodenal second portion invading the pancreatic head and inferior vena cava. (B) Follow-up CT scan 3 months after treatment with imatinib showed a remarkable reduction in the size of the tumor, to 4.4×3.2 cm. (C) Five months after the treatment, CT scan revealed a slightly reduction in the tumor size, to 4×2.8 cm. |
 | Fig. 2.Duodenoscopic finding showed that a huge mass with central ulceration at the ampulla of Vater. |
 | Fig. 3.Endoscopic retrograde chol-angiographic findigs. (A) Cholan-giogram showed a metallic stent (10×4 cm), which was placed at the distal common bile duct. (B) The stent was seen at the ampulla with ulcerative mass on endoscopic view. |
 | Fig. 4.Follow-up duodenoscopic finding 3 months after treatment with imatinib showed that the previously noted nodular mass around metal stent was almost disappeared and only small nodular lesion with granular surface was seen around the im-beded stent opening. |
 | Fig. 5.(A) Gross finding of the resected tumor showed submucosal whitish mass-like lesion with focal myxoid appearance (3×1.8×2 cm) in the ampulla of Vater, which was extending into the pancreas parenchyme. (B) Microscopic findings revealed well circumscribed lobulated tumor tissue, consisted of interlacing or whorling of spindle cells with intervening hyalinized stroma. It showed moderate to high cellularity and mitotic figures were rarely seen ＜5/10HPFs (H&E, ×400). (C, D) Immunohistochemical stainings showed positivity for CD 34 and CD 117. |
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