2 Hemangioblastoma Epidemiology M:F is 2:1 Age: 30 - 50 Uncommon (1-3% of all intracranial neoplasms)Most common intraaxial, nonmetastatic posterior fossa tumor in adultsSometimes referred to as Lindau Tumors
4 Hemangioblastoma Sporadic (75%) Von Hippel Lindau Disease (25%) VHL geneTumor suppressor gene3p25Autosomal Dominant Inheritance PatternCafé au lait spotsTumorsCNS HemangioblastomasPheochromocytomaRetinal AngiomasClear Cell Renal CarcinomaRenal CystsPancreatic Islet Cell TumorsPancreatic CystsExtremely high probability of developing a second mutation in at least 1 cell in the body.
6 Hemangioblastoma Pathology Appearance Benign vascular neoplasm WHO grade ISubtypes: Reticular and CellularArises from hemangioblastsAppearanceCherry RedCystic filled with clear fluidAttached to the pia (rich vascular supply)
7 Hemangioblastoma No grading or staging system Workup MRI of the brain and spineCT Abdomen - evaluate the kidneys, pancreas, adrenalsOpthalmology consultAngiography (may help the surgeon plan their approach)
9 HemangioblastomaA) Schematic representation of the distribution of CNS hemangioblastomas (red dots) in the 25 von Hippel-Lindau disease patients on MRI. Most (98%) of hemangioblastomas were found below the level of the tentorium in the cerebellum, brainstem, and spinal cord.(B–D) Contrast-enhanced MRI demonstrating representative locations of hemangioblastomas including the cerebellum (B), brainstem (C) and spinal cord (D). (B) Axial view through the cerebellum demonstrating a hyperintense enhancing hemangioblastoma (arrow) with surrounding edema (hypointense area surrounding the tumor) that frequently is associated with these lesions. (C) Sagittal view through the posterior fossa demonstrating a hyperintense enhancing brainstem (medullary) hemangioblastoma (arrow) with surrounding edema. (D) Sagittal view through the thoracic and lumbar spinal cord demonstrating two hyperintense enhancing hemangioblastomas (arrows). The superior tumor is associated with a large intraspinal cyst (syrinx) that is common with these neoplasms (arrowhead)
11 Hemangioblastoma Other Treatment Options Endovascular Embolization Antiangiogenic TherapyAdvantage of SRSAlpha/Beta ratio for HB is thought to be close to that for normal responding tissue. Therefore, hypofractionated radiation is advantageous.
12 Hemangioblastoma Outcomes Generally curable with surgery Local recurrence after surgery is higher with the following:VHL SyndromeMultiple HemangioblastomasYounger AgeCellular HistologyCellular has a % recurrence rateReticular has a % recurrence rateSubarachnoid dissemination is rare.
13 Retrospective Review Stanford University 1991 - 2007 Neurosurgery Vol. 65, No. 1, p. 79, 2009Retrospective ReviewStanford University92 lesions in 31 pts26 pts had VHLAll treated with SRSMean patient age: 41Dose: Gy [Ave Gy]Ave. tumor volume: 1.8 cm3
15 Median follow up of 69 months 16% progressed22% regressed62% stableLocal control rates36 months: %60 months: %Lesion-associated symptoms improved in 36 / 41 tumors5 patients developed radiation necrosisAuthors concluded that SRS is safe and effective in the treatment of HBs and is an attractive alternative to surgery for patients, including those with VHL disease.