No abstract

A case of Von Recklinghausen's disease with duodenal somatostatinoma is reported. A search of the world's literature revealed 27 patients with Von Recklinghausen's disease associated with an immunohistologically proved duodenal somatostatinoma. Twenty-nine cases of duodenal somatostatinoma not associated with Von Recklinghausen's disease and 32 cases of pancreatic somatostatinomas have been identified for comparison. While their histology may be similar in many respects, the duodenal and pancreatic somatostatinomas show significant differences, especially in hormonal and growth behaviors. In contrast to its pancreatic counterpart, the duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, is seldom associated with a recognizable "somatostatin syndrome," often contains psammoma bodies, and is less frequently associated with demonstrable metastases at the time of operation.

Metastasizing cells frequently bypass the initial filters in lymph nodes, liver, or lung to become established in secondary or tertiary sites. Intrapancreatic metastases or multicentric tumors also may develop more frequently than generally has been recognized. Small cancers (< 2 cm in diameter) are often associated at autopsy with remote metastases. These facts would appear to limit the usefulness of the current staging of resected cancers of the pancreas. Cancers of the body or tail are characterized by transperitoneal and hematogenous spread to a greater extent than are those of the head. Anatomical studies often do not explain the cause or the extent of ascites associated with pancreatic adenocarcinoma. As previously indicated, chronic pancreatitis appears to be further confirmed as a precursor of pancreatic cancer.