Somatostatinoma of the duodenum

Abstract

An adenocarcinoma of the second portion of the duodenum in a 26-year-old male is presented. The patient was suffering from pain in the epigastrium. Immunofluorescent studies revealed that it consisted almost exclusively of cells with a distincly positive somatostatin-like immunoreactivity. Ultrastructurally, the cytoplasm of the tumor cells had numerous large round granules (about 400 micrometers) with variable electron density. Most of these cells closely resembled the D cells normally seen in the duodenum and the islets of the pancreas, although a few argyrophil cells could be demonstrated by light microscopy. Radioimmunoassay of extracts of the tumor revealed a large amount of somatostatin (2260 pg/mg); substance P and VIP were detected also. Somatostatinoma has been known to occur in the pancreas, but this seems to be the first somatostatinoma found in the intestine.

Somatostatinoma syndrome: a challenging differential diagnosis among pancreatic tumors

“…The unmotivated patient only sought treatment because she was being taken care of by neighbors, reaching the medical facility Somatostatinoma derive from the somatostatin-producing delta cells of the pancreas or the endocrine cells of the digestive tract 5,6 and may be sporadic (93.1%) or familial (6.9%) in association with neurofibromatosis type 1 (NF1), multiple endocrine neoplasia type 1 (MEN1), and Von Hippel-Lindau syndromes. 4,[7][8][9][10] Most somatostatinomas (56%) originate in the pancreas and are usually functional.…”

A large functional somatostatinoma in the pancreatic tail: Atypical CT appearances

“…They derive from the somatostatin-producing delta cells of the pancreas or the endocrine cells of the digestive tract (6, 7) and may be sporadic (93.1%) or familial (6.9%) in association with neurofibromatosis type 1 (NF1), multiple endocrine neoplasia type 1 (MEN1), and Von Hippel-Lindau syndromes (8)(9)(10)(11). The estimated annual incidence is 1 in 40 million (12), with a median age at onset of 54 years.…”

Incidental Small Ampullary Somatostatinoma Treated with Ampullectomy 2 Years After Diagnosis

“…About 70% of all somatostatinomas originate in the pancreas [13], arising from the D-cells of Langerhans' islets [14]. D-cells are present in all the gastrointestinal tract, and extrapancreatic somatostatinomas have been described arising in the duodenum, in the jejunum, in the cystic duct and in the rectum [4][5][6][7][8].…”

“…Seventy percent occur in the pancreas, with the remaining ones arising from the D-cells of duodenum [4], ampulla [5], jejunum [6], cystic duct [7], or rectum [8]. Pancreatic somatostatinomas are often malignant (90%), with hepatic or lymph node metastases in 75% of cases at the time of diagnosis [9]; extrapancreatic somatostatinomas are often smaller and have a better prognosis [10].…”