[Pancreatic island cell tumors and their syndromes. I. Insulinomas, organic hypertinsulinism].

Based on a retrospective investigation of 61 surgical and autopsy specimens, the histopathologic features and clinical manifestations of islet cell tumors are described. In 33 cases reviewed in the first part of this study there existed indications of primary hyperinsulinism (HI) which was generally verified through fast test, stimulation tests or by insulin determination. In 25 cases the HI was caused by benign B-cell adenomas and in 3 cases by metastasizing carcinomas. In another case, polymacronesia of the pancreas with all patterns from abnormally structured islets to true adenomas was found. This patient was free of symptoms after subtotal pancreatectomy. In 4 other cases neither adenomas nor evident islet changes were identified; subtotal pancreatectomy cured only one patient. The insulinomas were surgically removed in 16 cases locally and in 9 cases by partial pancreatectomy. In two patients the adenomas were detected only after a second operation, and a third suffered from HI despite removal of two adenomas. The majority of insulinomas were characterized by staining and immunohistological technique, and a few electronmicroscopically. Two types of differentiation could be distinguished. The symptomatology of the three carcinoma patients was complex. Two of these patients suffered from severe ulcer disease reminiscent of Zollinger-Ellison syndrome. The third patient fell ill with erythema and other symptoms known from the "glucagonoma syndrome". These malignant tumors could only be incompletely characterized by staining, immunohistology and electronmicroscopy.