Article

Pancreatic GIST with pancreas divisum: A new entity.

Division of Digestive Surgery, Oujda CHU, University Mohammed First, Oujda, Morocco. Electronic address: .
International journal of surgery case reports 09/2012; 4(1):68-71. DOI: 10.1016/j.ijscr.2012.09.007
Source: PubMed

ABSTRACT INTRODUCTION: Gastrointestinal stromal tumours (GISTs) are uncommon intra-abdominal tumours. These tumours tend to arise with a higher frequency in the stomach and the small bowel. In fewer than 5% of cases, they originate primarily from extra-gastrointestinal tumours (EGISTs). Gastrointestinal stromal tumour of the pancreas is very rare. Only few cases have been published. We report the first case of stromal tumour of the pancreas with concomitant pancreas divisum. PRESENTATION OF CASE: A 39-year-old male who presented with constipation and abdominal pain. A computerized tomography demonstrated a 9cm pancreatic mass, without liver lesions. A Whipple procedure with segmental colectomy was performed with success. After 24months follow up, the patient is doing well and disease-free. DISCUSSION: Mesenchymal tumours of the pancreas are extremely rare, accounting for less than 1% of all pancreatic tumours. The endoscopic ultrasound is helpful for diagnosis. Surgical resection with negative pathologic margins remains the treatment of choice. CONCLUSION: in our knowledge, this is the first case of pancreatic GIST with pancreas divisum. Although pancreatic GISTs are uncommon tumours, they must be considered in the differential diagnosis of solid pancreatic lesions. Even though the tumour can be evaluated as high risk, treatment must be aggressive in order to improve survival rate.

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    ABSTRACT: Primary extra-gastrointestinal stromal tumor (EGISTs) arising in the pancreas is extremely rare: only 20 cases have previously been reported in the English literature from 2000 to 2013. We reported a case of EGIST of the pancreas in a 69-year-old woman who presented with abdominal pain and with a solid, heterogeneously enhancing neoplasm in the uncinate process of the pancreas, revealed preoperatively by an abdominal computed tomography scan. A diagnosis of neuroendocrine tumor was suggested. Positron emission tomography with 68Ga-DOTATOC did not show pathological accumulation of the tracer in the pancreas. The patient underwent enucleation, under ultrasonic guidance, of the pancreatic tumor that emerged to the surface of the pancreas. Histopathology and immunohistochemical examination confirmed the final diagnosis of EGIST of the pancreas (CD117+), with one mitosis per 50 high-power fields. Although rarely, GIST can involve the pancreas as a primary site, and this tumor should be considered in the differential diagnosis of pancreatic neoplasms.
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