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Carcinoid Syndrome, Acromegaly, and Hypoglycemia Due to an Insulin-Secreting Neuroendocrine Tumor of the Liver

Department of Internal Medicine, Medical Policlinic (J.F., A.H., P.W.), Department of Pathology (P.K.), and Department of Radiology (T.P.), University Hospital of Zurich, CH-8091 Zurich; and Institute of Pathology (P.K.), Kantonsspital, CH-5404 Baden, Switzerland

Address correspondence and requests for reprints to: Jörg Furrer, M.D., Department of Internal Medicine, Medical Policlinic, University Hospital of Zurich, CH-8091 Zurich, Switzerland.

We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor’s humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor I due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor I and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and C-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation.