Atypical localisation of a gastrointestinal stromal tumour: a case report of pancreas gastrointestinal stromal tumour

Abstract

An atypically localized gastrointestinal stromal tumor: a case report of pancreas gastrointestinal stromal tumor Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. They can arise from any part of the tract as well as the omentum, mesentery or retroperitoneum. In this study, we present a case of a GIST originating from an extraordinary site, the pancreas. Evaluation of 30-year-old man with complaints of abdominal distension revealed a cystic, distal pancreatic lesion 13 cm in diameter. There were no intra-abdominal or distant organ metastases. The patient was operated with a diagnosis of cystic pancreas tumor, distal pancreatectomy and splenectomy was performed. The lesion was diagnosed as gastrointestinal stromal tumor upon histopathological examination. He was discharged on the sixth postoperative day without any complications and is being followed up for 21 months without systemic or local recurrence. Extragastrointestinal GISTs are reported rarely. To our knowledge, only one pancreatic GIST has been reported previously in the English literature.Key Words: Gastrointestinal stromal tumour, extragastrointestinal, pancreas INTRODUCTIONGastrointestinal stromal tumors (GIST) are the most common tumors of mesenchymal tissue origin in the gastrointestinal tract (GI) (1, 2). It can be seen anywhere throughout the GI tract from the oesophagus to the rectum, but the most common location is the stomach and jejunum (1, 2). Due to mutations of some tyrosine kinase receptors like C-kit and platelet derived growth factor receptor alpha oncogene (PDGFRA), it arises from Cajal cells that provide connection between the myenteric layer and the muscular layer in the GI tract (3-6). Tumors originating from omentum , mesentery, or retroperitoneum, with similar clinical and pathological findings have been described, and these stromal tumors not originitanig from the digestive system are classified as extra-gastrointestinal stromal tumors (EGIST) (7-9). Rare cases of EGIST arising from the gall bladder, abdominal wall, perivesical area, pharynx, posterior mediastinum, liver and pancreas have also been reported (9, 10). In this case report, we present a GIST that did not arise from the gastrointestinal tract (extragastrointestinal), but the pancreas. CASE PRESENTATIONA thirty-year-old male patient was seen to have a 13 cm mass lesion in the distal pancreas on abdominal ultrasonography performed in another center during evaluation of his abdominal distention. A thoraco-abdominal computed tomography (CT) scan revealed a cystic mass that was 13 cm in size, originating from the distal pancreas, located behind the stomach without any evidence of gastric invasion and containing areas of necrosis and septa (Figure 1). Neither intra-abdominal spread nor distant metastases were observed. Tumor markers including Carbonic antigen (CA) 19-9, carcinoembryonic antigen (CEA) and neuron-specific enolase (NSE) were normal. Considering factors including the patient's young age, normal levels o...

Pancreatic extragastrointestinal stromal tumor invading the duodenum

“…GISTs that originate from the soft tissues of the abdomen and retroperitoneum are called extragastrointestinal stromal tumors (EGISTs). EGISTs that arise from the pancreas are extremely rare, and only 15 cases have been previously reported (7)(8)(9). Here we report a case of a large pancreatic EGIST that invaded the duodenum.…”

“…a hematopoietic progenitor cell antigen, whereas 20%-30% are positive for SMA and 10% are positive for the S-100 protein. Approximately 40%-60% of GISTs occur in the stomach, whereas 30%-40% occur in the small intestine, 10% in other parts of the gastrointestinal tract, and 5% elsewhere in the abdominal cavity (3,5,(8)(9)(10)(11)(12)(13)(14). GISTs are infrequent neoplasms.…”

An Extragastrointestinal Stromal Tumor Originating from the Pancreas

“…These tumors most commonly occur in the stomach, with a frequency of 60–70% of all GISTs, followed by 20–25% in the small intestine, 5% in the colon and rectum, and < 5% in the esophagus [ 4 ]. Rarely, GIST occurs in the liver, gallbladder, pancreas, and bladder, and is called extragastrointestinal stromal tumor (EGIST), which accounts for < 5% of all GISTs [ 5 , 6 ]. A previous report indicated that < 5% of EGIST cases occur in the pancreas [ 7 ].…”

Pancreatic gastrointestinal stromal tumor: A case report

“…It is reported that, EGISTs are extremely rare, represent 5%-10% of all GISTs [7], [8]. EGISTs are frequently located in the omentum, mesentery or retroperitoneum [9].…”