[Recurrent and metastasizing hemangiopericytoma of the meninges with paraneoplastic hypoglycemia].
Primary meningeal hemangiopericytomas (formerly referred to as angioblastic meningiomas) are by most authors no longer considered to be actual meningiomas but rather thought to be intracranial hemangiopericytomas. Their biological behavior is usually malignant, with recurrences and metastases, often at intervals of years. Both intracranial and extracranial hemangiopericytomas 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 recurrencies 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 endocrine 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 macromolecular share. The observed paraneoplastic hypoglycemia is probably brought about by coincidence of blocked hepatic glucose production, suppressed lipolysis and increased peripheral glucose uptake. Autopsy revealed 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 showed the characteristics features of a hemangiopericytoma. Light microscopic pictures of the primary tumor, recurrences and metastases were identical. Additional autopsy findings were a papillary carcinoma of the right kidney, an angiomyolipoma of the left kidney and a thecoma of the left ovary.
Wegmann
W
W
Kantonales Institut für Pathologie, Liestal.
Vonesch
H J
HJ
Kamber
J
J
Kiss
D
D
D000368:Aged; D005260:Female; D006393:Hemangiopericytoma / Q000150:complications / Q000473:pathology*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology*; D007328:Insulin / Q000378:metabolism; D000078790:Insulin Secretion; D007334:Insulin-Like Growth Factor I / Q000096:biosynthesis; D008099:Liver / Q000378:metabolism; D008113:Liver Neoplasms / Q000556:secondary; D008577:Meningeal Neoplasms / Q000150:complications / Q000473:pathology*; D009364:Neoplasm Recurrence, Local; D010257:Paraneoplastic Syndromes / Q000150:complications*
D007328:Insulin; D007334:Insulin-Like Growth Factor I
Rezidivierendes und metastasierendes Hämangioperizytom der Meningen mit paraneoplastischer Hypoglykämie.