hemangioblastoma. epidemiology m:f is 2:1 age: 30 - 50 uncommon (1-3% of all intracranial...

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%