PANCREATIC SOMATOSTATINOMAS AND NEM-1 - R EPORT ON ONE OBSERVATION - LITERATURE-REVIEW

The pancreatic somatostatinoma belongs to the type of rare endocrine t umors of the pancreas. We report the observation of a 54 year old woma n. Previously she was suffering from diabetes mellitus. An abdominal u ltrasonography revealed an endocrine tumor of the pancreatic tail. The re was no specific symptomatology with the exception for the hyperglyc aemia. The diagnosis of somatostatinoma was certified post operatively by the immunocytochemistry of the turner. Then, the patient developed a hypercalcaemia associated with an increase of parathyroid hormone. The surgery of the neck revealed three hyperplastic parathyroids, indu cing this association as a multiple endocrine neoplasia type I (MEN I) . The patient did not develop pituitary tumor Afterwards, scintigraphy with 111 Indium- octreotide showed a residual tumor at the head of pa ncreas. Basal levels of somato-statine and calcium, pentagastrine test , computed tomography scan, arteriography were negative. The presence of a second somatostatinoma was confirmed by surgery and immunohistolo gy. One year after the surgery, the patient remains clincally well. Th e pancreatic localization of the somatostatinoma in a MEN I is poorly documented. Its malignant nature can only be assured by the presence o f metastases. The genetic detection of the MEN I becomes possible. Abo ve all, the treatment is based on surgery and/or chemotherapy (Fluoro- Uracile; Streptozotocine). In our-case, 111 In-octreotide scintigraphy was the only method demonstrating a residual focus, suggesting it cou ld be an element of reference for the diagnosis and survey of somatost atinoma the watch of patients having a treatment for somatostatinoma.