Supratentorial haemangioblastoma not associated with Von Hippel Lindau complex or polycythaemia: case report and literature review

Abstract

Solitary or multiple supratentorial haemangioblastomas are rare and may be associated with Von Hippel Lindau complex (VHLC), polycythaemia or a family history of VHLC. An extensive review of the literature (1902-1992) has revealed 112 cases. They account for 2-8% of all haemangioblastomas and are commonly intraparenchymatous (86.8%) and infrequently intraventricular (6.6%). However, a meningeal location 5.6%) is extremely rare. Only one case of a solitary leptomeningeal haemangioblastoma has previously been reported and this was associated with a family and personal history of VHLC, cerebellar haemangioblastoma and renal carcinoma. We report a solitary solid left parietal leptomeningeal haemangioblastoma mimicking a meningioma or a secondary deposit on computed tomography in a 72-year-old male. There were no manifestations of or a family history of VHLC or polycythaemia.

Brainstem hemangioblastoma and sellar mass

“…[2] Supratentorial Hb is rare (4–11%),[2] amongst which intraparenchymal are 86.8%, intraventricular are 6.6%, and meningeal are 5.6%. [3] The incidence of sellar Hb varies from <1–29% amongst the supratentorial Hbs. [3] Supratentorial and spinal tumors are usually completely solid compared to the cystic solid variety in the cerebellum.…”

“…[3] The incidence of sellar Hb varies from <1–29% amongst the supratentorial Hbs. [3] Supratentorial and spinal tumors are usually completely solid compared to the cystic solid variety in the cerebellum. Solid tumors comprise 20–29% and are associated with poorer outcome and higher recurrence compared to cystic tumors.…”

Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report

“…Based on previous studies, the occurrence of supratentorial HBLs is thought to be in the range of 2% to 8% of all HBLs [3,4,6], accounting for 116 reported cases from 1902 to 2004 [5]. Supratentorial tumors were mostly found in the frontal, parietal or temporal lobes [7].…”

Congenital Cystic Hemangioblastomas of the Cerebral Hemisphere in a Neonate without Alteration in the <i>VHL</i> Gene

“…18]. Supratentorial hemangioblastomas appear more common in sporadic, non-vHL cases, but almost all tumors have been identified in adults [1, 2, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22]. Most supratentorial hemangioblastomas in patients of any age reported in the literature antedate the identification of the VHL gene.…”