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Ectopic Bioactive Luteinizing Hormone Secretion by a Pancreatic Endocrine Tumor, Manifested as Luteinized Granulosa-Thecal Cell Tumor of the Ovaries

Departments of Endocrinology and Diabetes (G.P., A.A., N.M., T.K., G.Ka.), Pathology (G.Ko.), and Surgery (K.V.), G. Gennimatas General Hospital, 115 27 Athens, Greece; Department of Pathology and Laboratory Medicine, Mayo Clinic Foundation (R.V.L.), Rochester, Minnesota 55905; and Department of Pathology, St. Michael’s Hospital (G.Ko., E.H., K.K.), University of Toronto, Toronto, Ontario, Canada M5B 1W8

Address all correspondence and requests for reprints to: Dr. George P. Piaditis, Department of Endocrinology and Diabetes Center, KOFKA Building, 1st Floor, G. Gennimatas General Hospital of Athens, 154 Messogion Avenue, 115 27 Athens, Greece. E-mail: edk-pgna{at}otenet.gr.

Endocrine pancreatic tumors are rare neoplasms consisting of multipotent cells capable of secreting various bioactive substances causing characteristic clinical syndromes. Ovarian stromal hyperthecosis is characterized by varying degrees of luteinized stromal cell proliferation after sustained LH and/or human chorionic gonadotropin stimulation, clinically manifested by symptoms/signs of virilization resembling the polycystic ovary syndrome (PCOS). We report a case of ectopic bioactive LH production from a pancreatic endocrine tumor in a 33-yr-old woman with rapidly developing symptoms/signs of hyperandrogenism and markedly elevated serum androgen and LH levels leading to hyperthecosis and bilateral luteinized granulosa-thecal cell tumors of the ovaries. Although the patient was initially thought to have either severe PCOS or an LH-secreting pituitary tumor, an LH-producing pancreatic endocrine tumor bearing somatostatin receptors was demonstrated on scintigraphy with [¹¹¹In]octreotide and abdominal imaging. Symptoms and signs of hyperandrogenism resolved after the resection of the tumor. Immunohistochemistry, in situ hybridization, and electron microscopy studies confirmed LH synthesis by the tumor cell. Although extremely rare, ectopic LH production from nonpituitary endocrine tumors should be considered in the differential diagnosis of hyperandrogenism, particularly when associated with highly elevated serum LH levels.