1. Hemangioblastoma

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 adults
Sometimes referred to as Lindau Tumors

3. Hemangioblastoma
Arvid Lindau

4. 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.

5. Hemangioblastoma Location Symptoms 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

6. Hemangioblastoma Pathology Appearance 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)

7. 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)

8. Hemangioblastoma

9. 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.
(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)

10. Hemangioblastoma

11. 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.

12. 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 % recurrence rate
Reticular has a % recurrence rate
Subarachnoid dissemination is rare.

13. Retrospective Review Stanford University 1991 - 2007
Neurosurgery Vol. 65, No. 1, p. 79, 2009
Retrospective Review
Stanford University
92 lesions in 31 pts
26 pts had VHL
All treated with SRS
Mean patient age: 41
Dose: Gy [Ave Gy]
Ave. tumor volume: 1.8 cm3

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

15. Median follow up of 69 months
16% progressed
22% regressed
62% stable
Local control rates
36 months: %
60 months: %
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.

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

17. Neurosurgery 63: , 2008
Retrospective review
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

23. IJROBP, Vol. 69, No. 5, 2007
Retrospective Review, , PMH
18 patients (5 VHL; 13 sporadic); 31 lesions
Doses ranged from Gy; Parallel opposed fields
Median follow up: 5.1 years
DFS at 5 and 10 years was 57% and 30%