Somatostatin-producing duodenal carcinoma: Clinicopathological description of a case

Abstract

Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. They are seldom associated with typical clinical symptoms. Their diagnosis is confirmed only by histological and immunohistochemical studies and the presence of specific hormones. Two distinct clinicopathological forms of somatostatinoma exist: duodenal and pancreatic somatostatinomas. Clinically, compared to pancreatic somatostatinomas, duodenal somatostatinomas are more often associated with nonspecific symptoms and neurofibromatosis, but less often with somatostatinoma syndrome or metastasis. We report a case of somatostatin-producing duodenal carcinoma in a 45-year-old female with neither neurofibromatosis nor somatostatinoma syndrome. Abdominal computed tomography showed a 18 mm mass in the duodenum which had given rise to multiple lymph node metastases. Although the endoscopic biopsies were free of malignancy, the patient subsequently underwent Whipple's operation for the duodenal mass. Immunohistochemical analysis confirmed the diagnosis of somatostatin-producing carcinoma.

Pancreatic Neuroendocrine Tumors: A Literature Review

“…These tumors originate from the D cells of the apud system (endocrine cells) can develop in the tissues of the pancreas and duodenum. About 56%-70% of somatostatinomas are from pancreatic tissues, 36% in the head of the organ, 14% in the body, and 32% in the pancreatic tail [27]. Somatostatinomas can be functional or non-functional NETs.…”

“…Around 40% of the metastases are hepatic and 30% in the lymph nodes. Pancreatic somatostatinomas are most often larger than duodenal ones, with an average size ranging between 0.3 and 6cm [27,30]. The duodenal somatostatinomas are commonly associated with nonspecific symptoms, with neurofibromatosis and less frequently with somatostatinoma syndrome or metastasis [27].…”

“…Pancreatic somatostatinomas are most often larger than duodenal ones, with an average size ranging between 0.3 and 6cm [27,30]. The duodenal somatostatinomas are commonly associated with nonspecific symptoms, with neurofibromatosis and less frequently with somatostatinoma syndrome or metastasis [27]. Symptoms are not typical and can be confounded with other diseases such as diabetes, indigestion, and cholelithiasis since when the tumor is secretory, patients have somatostatinoma syndrome, characterized by diabetes, gallstones, and steatorrhea, which are similar to other diseases.…”