RECURRENT AND METASTASIZING MENINGEAL HEM ANGIOPERICYTOMA ASSOCIATED WITH PARANEOPLASTIC HYPOGLYCEMIA

Primary meningeal hemangiopericytomas (formerly referred to as angiobl astic meningeomas) are by most authors no longer considered to be actu el meningeomas but rather thought to be intracranial hemangiopericytom as. Their biological behaviour is usually malignant, with recurrences and metastases, often at intervals of years. Both intracranial and ext racranial haemangiopericytomas may, however rarely, be accompanied by paraneoplastic hypoglycemias. Our own characteristic observations are based on a female patient aged 67 at the time of her death, in whom a meningeal tumor was resected first at the age of 41. Later recurrencie s were removed at the age of 52 and 58 respectively. Three years prior to her death liver metastases had developed followed by increasingly frequent attacks of early morning hypoglycemia with blood sugar levels ranging between 1.4-2.3 mmol/l. Specific examinations revealed low en docrine production of insulin and a distinctly decreased insulin-like growth factor (IGF) I of 25 ng/mb (normal 120-130) and a normal value of total IGF II of 724 ng/ml (normal 400-900), though with a big macro molecular share. The observed paraneoplastic hypoglycemia is probably brought about by coincidence of blocked hepatic glucose production, su ppressed lipolysis and increased peripheral glucose uptake. Autopsy re vealed a third intracranial recurrence of meningeal hemangiopericytoma and a large metastatic liver. No other sites of metastases were found . Histologic, immunohistologic and electron microscopic findings showe d the characteristics features of a hemangiopericytoma. Light microsco pic pictures of the primary tumor, recurrences and metastases were ide ntical. Additional autopsy findings were a papillary carcinoma of the right kidney, an angiomyolipoma of the left kidney and a thecoma of th e left ovary.