Somatostatinomas are rare, malignant,
somatostatin-producing
neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of
Von Recklinghausen's disease and duodenal
somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with
Von Recklinghausen's disease and a
tumor of the pancreatic head that was diagnosed due to painless
icterus. Histopathological examination after pylorus-preserving
pancreatoduodenectomy revealed the existence of a duodenal
somatostatinoma with
lymph node metastases. Characteristics of the association of
von Recklinghausen's disease and
somatostatinoma, and
therapy and prognosis will be discussed. In patients with
Von Recklinghausen's disease and an ampullary
tumor, a
somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal
somatostatinoma is frequently associated with
Von Recklinghausen's disease, often contains psammoma bodies, is rarely associated with a recognizable "
somatostatin syndrome", and is hardly ever associated with demonstrable
metastases at the time of diagnosis. Small
tumors arising in the duodenum may be treated with local excision, whereas larger
tumors should be treated by total excision, which may entail a partial
pancreatoduodenectomy.