1. Hemangioblastoma
Intern 蔡佽學

2. Information Name: 李x珠 Sex: female Age: 49
C.C: Headache and vomiting was noted for half year

3. Brief history Headache and dizziness was noted for 8months.
In about 3-4 months ago, nausea/vomiting were also noted.
In 市立H., brain MRI was done and showed hydrocephalus and about 2*2 mass on right hemicerebellum.

4. PE/NE: no specific findings
Family history: non-contributory
Lab: hyperglycemia

5. MRI

8. Discussion

9. Introduction Benign, slowly growing vascular neoplasm
4 % of all intracranial neoplasms
most common primary intraaxial tumor in adult posterior fossa
sporadically or associated with von Hippel-Lindau (VHL) disease (25%)

10. Age
adulthood (>80%): years,
average age of 33 years; M > F
childhood (<20%): in von Hippel-Lindau
disease (25%); girls
Location: paravermian cerebellar hemisphere > spinal cord > cerebral hemisphere / brainstem
Multiple lesions in 10%

11. VHL disease
An autosomal dominant disorder caused by a deletion in chromosome 3 (3p)
Mutation of tumor supressor gene
 overexpression of VEGF
 highly vascularized tumors
occurs in 1:36-45,000 population

13. Symptoms Depend on the site of the hemangioblastoma Infratentorial:
Headache is the most frequent
NE is often normal or shows cerebellar signs
Spinal : Paresthesia and/or pain
Sensory or motor loss
Brainstem: orthostatic hypotension.

14. Hydrocephalus due to obstruction of fourth ventricle from mass effect
Classic polycythemia in 20% of cerebellar hemangioblastomas.
Subarachnoid or intra-axial hemorrhage occur in only a few cases
Hormone-mediated growth phenomenon

15. Pathology Two cell populations: Endothelial cells
- dense network of small vascular
channels with thin lining
Stromal cells
- exhibit some hyperchromasia and pleomorphism
- classically lipidized features
- usually no necrosis or mitotic activity
- well-dermacated and not infiltrative

16. A classic picture of hemangioblastoma in situ, with engorged, numerous arteries and draining veins leading to and from a well-circumscribed and highly vascular tumor visible at the pial surface.

18. Image MRI is much more sensitive than CT
CT: Cystic sharply marginated mass with peripheral mural nodule with homogeneous enhancement Solid with intense homogeneous enhancement

20. MRI
hypointense areas on T1WI + hyperintense areas on T2WI in cyst formation
well-demarcated tumor mass moderately hypointense on T1WI + T2WI
heterogenous enhancement after injection of Gd-TPA

21. Angio:
densely stained tumor nidus within cyst
staining of entire rim of cyst
draining vein

22. T1-weighted Gd-TPA MRI

23. T1-weighted Gd-TPA MRI of cystic hemangioblastoma

24. Differential diagnosis
Cystic astrocytoma >5 cm, calcifications, larger nodule, thick-walled lesion, no angiographic contrast blush of mural nodule, no erythrocythemia
Arachnoid cyst (if mural nodule not visualized)
Metastasis (more surrounding edema)

25. Cystic astrocytoma Enhancement of cyst wall
No enhancement of cyst wall

26. Treatment Surgery may be curative in sporadic cases
For large lesions, embolization of feeding arteries is typically performed
Timing : tumor growth, neurologic deficit, or hemorrhage

27. Stereotactic radiosurgery neurologically sensitive area multiple tumors
Conventional fractionation RT

28. Prognosis Low recurrence risk if total resection
Worse prognosis in VHL
Need to screen for mutation if young person with hemangioblastoma
VHL :
Median life expectancy is 49 years
Death usually related to CNS hemangioblastoma or renal cell carcinoma

29. Thanks