Hemangioblastoma

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Hemangioblastoma
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Hemangioblastoma

• Epidemiology
• MF is 21
• 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

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Hemangioblastoma
Arvid Lindau
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Hemangioblastoma

• 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.
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Hemangioblastoma

• 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

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Hemangioblastoma

• 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)

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Hemangioblastoma

• 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)

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Hemangioblastoma
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Hemangioblastoma
A) 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. (BD) 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)
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Hemangioblastoma
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Hemangioblastoma
Other Treatment Options Endovascular
Embolization Antiangiogenic 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.
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Hemangioblastoma

• 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.

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• 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

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• Cerebellum 52
• CPA 4
• Thalamus 1
• Brainstem 9
• Cervical Cord 8
• Thoracic Cord 7
• Lumbar Cord 1

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• 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.

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• Neurosurgery Vol. 65, No. 1, p. 79, 2009

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• Neurosurgery 63443-451, 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

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• 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

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