Carcinoid syndrome in a child presenting with pancreatic somatotropinoma

Abstract

Carcinoid syndrome develops as a sequel of enhanced secretion of vasoactive substances from neuroendocrine tumours (NET). Somatostatinoma is an extremely rare variant of NET that manifests itself in cholelithiasis, steatorea, and diabetes mellitus. Somatostatinoma is usually diagnosed in adult subjects. Carcinoid syndrome is not a typical consequence of somatostatinoma. We present the first case report of somatostatinoma that was diagnosed in a child having carcinoid syndrome. The diagnosis was made difficult by the negative results of the search for biochemical markers of carcinoid syndrome and the small size of the primary tumour that could not be visualized by computed tomography. The immunohistochemical studies demonstrated secretion of somatostatin and calcitonin. The treatment of the child with somatostatin analogs proved inefficient which can be accounted for by the late diagnosis of the disease and the extensive metastatic process.