hemangioblastoma. epidemiology m:f is 2:1 age: 30 - 50 uncommon (1-3% of all intracranial...
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HemangioblastomaHemangioblastoma
HemangioblastomaHemangioblastoma
Epidemiology M:F is 2:1 Age: 30 - 50 Uncommon (1-3% of all intracranial neoplasms) Most common intraaxial, nonmetastatic posterior
fossa tumor in adults Sometimes referred to as Lindau Tumors
Epidemiology M:F is 2:1 Age: 30 - 50 Uncommon (1-3% of all intracranial neoplasms) Most common intraaxial, nonmetastatic posterior
fossa tumor in adults Sometimes referred to as Lindau Tumors
HemangioblastomaHemangioblastoma
Arvid Lindau
HemangioblastomaHemangioblastoma
Sporadic (75%) Von Hippel Lindau Disease (25%)
VHL gene Tumor suppressor gene 3p25
Autosomal Dominant Inheritance Pattern Café au lait spots Tumors
CNS Hemangioblastomas Pheochromocytoma Retinal Angiomas Clear Cell Renal Carcinoma Renal Cysts Pancreatic Islet Cell Tumors Pancreatic Cysts
Sporadic (75%) Von Hippel Lindau Disease (25%)
VHL gene Tumor suppressor gene 3p25
Autosomal Dominant Inheritance Pattern Café au lait spots Tumors
CNS Hemangioblastomas Pheochromocytoma Retinal Angiomas Clear Cell Renal Carcinoma Renal Cysts Pancreatic Islet Cell Tumors Pancreatic Cysts
Extremely high probability of developing a second mutation in at least 1 cell in the body.
HemangioblastomaHemangioblastoma
Location Cerebellum Spinal Cord Other
Brainstem Supratentorial Optic Nerves Peripheral Nerves Soft Tissues
Location Cerebellum Spinal Cord Other
Brainstem Supratentorial Optic Nerves Peripheral Nerves Soft Tissues
Symptoms Depend on location Brain / Posterior Fossa
Ataxia, discoordination Headache Subarachnoid hemorrhage
Spinal Cord Pain, weakness, numbness
Eye Visual Changes
Elevated Erythropoietin Secondary polycythemia
Symptoms Depend on location Brain / Posterior Fossa
Ataxia, discoordination Headache Subarachnoid hemorrhage
Spinal Cord Pain, weakness, numbness
Eye Visual Changes
Elevated Erythropoietin Secondary polycythemia
HemangioblastomaHemangioblastoma
Pathology Benign vascular neoplasm WHO grade I Subtypes: Reticular and Cellular Arises from hemangioblasts
Appearance Cherry Red Cystic filled with clear fluid Attached to the pia (rich vascular supply)
Pathology Benign vascular neoplasm WHO grade I Subtypes: Reticular and Cellular Arises from hemangioblasts
Appearance Cherry Red Cystic filled with clear fluid Attached to the pia (rich vascular supply)
HemangioblastomaHemangioblastoma
No grading or staging system Workup
MRI of the brain and spine CT Abdomen - evaluate the kidneys, pancreas, adrenals Opthalmology consult Angiography (may help the surgeon plan their approach)
No grading or staging system Workup
MRI of the brain and spine CT Abdomen - evaluate the kidneys, pancreas, adrenals Opthalmology consult Angiography (may help the surgeon plan their approach)
HemangioblastomaHemangioblastoma
HemangioblastomaHemangioblastomaA) 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)
HemangioblastomaHemangioblastoma
HemangioblastomaHemangioblastoma
Other Treatment OptionsEndovascular EmbolizationAntiangiogenic Therapy
Advantage of SRS Alpha/Beta ratio for HB is thought to be close to that for normal responding tissue. Therefore, hypofractionated radiation is advantageous.
HemangioblastomaHemangioblastoma
Outcomes Generally curable with surgery Local recurrence after surgery is higher with the
following: VHL Syndrome Multiple Hemangioblastomas Younger Age Cellular Histology
Cellular has a 20 - 25% recurrence rate Reticular has a 5 - 10% recurrence rate
Subarachnoid dissemination is rare.
Outcomes Generally curable with surgery Local recurrence after surgery is higher with the
following: VHL Syndrome Multiple Hemangioblastomas Younger Age Cellular Histology
Cellular has a 20 - 25% recurrence rate Reticular has a 5 - 10% recurrence rate
Subarachnoid dissemination is rare.
Neurosurgery Vol. 65, No. 1, p. 79, 2009
Retrospective Review Stanford University 1991 - 2007 92 lesions in 31 pts 26 pts had VHL All treated with SRS Mean patient age: 41 Dose: 12 - 40 Gy [Ave. 23.4 Gy] Ave. tumor volume: 1.8 cm3
Neurosurgery Vol. 65, No. 1, p. 79, 2009
Retrospective Review Stanford University 1991 - 2007 92 lesions in 31 pts 26 pts had VHL All treated with SRS Mean patient age: 41 Dose: 12 - 40 Gy [Ave. 23.4 Gy] Ave. tumor volume: 1.8 cm3
Cerebellum 52 CPA 4 Thalamus 1 Brainstem 9 Cervical Cord 8 Thoracic Cord 7 Lumbar Cord 1
Cerebellum 52 CPA 4 Thalamus 1 Brainstem 9 Cervical Cord 8 Thoracic Cord 7 Lumbar Cord 1
Median follow up of 69 months 16% progressed 22% regressed 62% stable
Local control rates 36 months: 85% 60 months: 82%
Median follow up of 69 months 16% progressed 22% regressed 62% stable
Local control rates 36 months: 85% 60 months: 82%
Lesion-associated symptoms improved in 36 / 41 tumors
5 patients developed radiation necrosis
Authors 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.
Lesion-associated symptoms improved in 36 / 41 tumors
5 patients developed radiation necrosis
Authors 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.
Neurosurgery Vol. 65, No. 1, p. 79, 2009
Neurosurgery 63:443-451, 2008
Retrospective review 1990 - 2006 University of Pittsburgh 32 patients; 74 tumors All received Gamma Knife SRS Median age: 44 13 pts had VHL (52 tumors) Median Dose: 16 Gy Median Volume: 0.72 ml Median follow up: 50 months
Neurosurgery 63:443-451, 2008
Retrospective review 1990 - 2006 University of Pittsburgh 32 patients; 74 tumors All received Gamma Knife SRS Median age: 44 13 pts had VHL (52 tumors) Median Dose: 16 Gy Median Volume: 0.72 ml Median follow up: 50 months
IJROBP, Vol. 69, No. 5, 2007 Retrospective Review, 1980 - 2004, PMH 18 patients (5 VHL; 13 sporadic); 31 lesions Doses ranged from 50.4 - 55.8 Gy; Parallel opposed fields Median follow up: 5.1 years DFS at 5 and 10 years was 57% and 30%
IJROBP, Vol. 69, No. 5, 2007 Retrospective Review, 1980 - 2004, PMH 18 patients (5 VHL; 13 sporadic); 31 lesions Doses ranged from 50.4 - 55.8 Gy; Parallel opposed fields Median follow up: 5.1 years DFS at 5 and 10 years was 57% and 30%
