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


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.

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.

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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Available from: Mehdi Soufi, May 21, 2015
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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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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the gastrointestinal tract that originate from the intestinal cells of Cajal (ICC) (Fletcher et al., 2002). Only a few cases have been described with extragastrointestinal stromal tumors (Kim et al., 2012; Soufi et al., 2013; Meng et al., 2011). They are often diagnosed as a pancreatic head tumor as they are very difficult to relate to the duodenum with CT, MRI, or ultrasound. We present a case of a sixty-four-year-old woman who presented with abdominal pain and cardialgia for a follow-up examination after breast cancer surgery. On laparotomy there was a 3 × 5 cm hypervascular mass arising from the pancreatic head with macroscopically no attachment to the duodenum. The patient underwent pancreatoduodenectomy (PD) modified after Traverso-Longmire, histopathology proved a duodenal GIST. This case proves that duodenal GISTs can grow invasively into the pancreas and appear as solid pancreas head tumor; therefore, these tumors should be included into differential diagnosis.
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    ABSTRACT: Aim: To provide an overview of the literature on pancreatic extragastrointestinal stromal tumors (EGISTs). Methods: We report a case of pancreatic EGIST and review published studies on pancreatic EGIST accessed via the PubMed, MEDLINE, Google Scholar, and Google databases. The keywords used were "pancreas and GIST", "pancreas and extra GIST", "pancreas and gastrointestinal stromal tumor", and "pancreas and extragastrointestinal stromal tumor". Literature reviews and/or duplicate studies were excluded. The search included articles published in the English language between January 1, 2000 and May 15, 2014. Results: From our literature survey, 30 manuscripts on pancreatic EGISTs were considered, of which 27 met the search criteria and three were excluded. The studies involved 30 patients (15 men, 15 women) with a mean age of 55.3 ± 14.3 years (range 30-84 years). The mean age of the male patients was 50.8 ± 13.7 years (range 30-84 years); that of the female patients was 59.9 ± 13.3 years (range 38-81 years). Tumor dimensions were obtained for 28 cases (mean 114.4 ± 78.6 mm; range 20-350 mm). Tumors were diagnosed incidentally in 23.3% of patients; abdominal discomfort and weight loss were the major complaints in symptomatic patients. Risk of aggressive behavior according to Fletcher criteria was determined in 25 of 30 patients (68%: high risk, 28%: intermediate risk, 4%: low risk). Histopathological examination revealed the presence of spindle cells in 96.1% of cases; CD117 and CD34 were present immunohistochemically in 96.6% and 84% of patients, respectively. The most common surgical procedures were distal pancreatectomy with splenectomy (n = 9) and pancreaticoduodenectomy (n = 7). The total follow-up period for the 28 patients ranged from 3-66 mo, during which locoregional or distant metastases were diagnosed in six patients and two patients died. Conclusion: Studies on EGISTs have only been published in the last decade. The lack of studies with large patient cohorts and long-term follow-up limits evidence-based commentary. In theory, each case should be assessed individually and further genetic and immunohistochemical studies are needed.
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