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capillary hemangioblastoma
Etiology:
- small number associated with von Hippel-Lindau disease
Epidemiology:
1) 7-10% of posterior fossa tumors
2) 4% of spinal cord tumors
Pathology:
- cerebellum most often involved (83%)
- also occurs in medulla (2%) & spinal cord (13%)
- occasionally multifocal
- often associated with or within a cyst & connected to meninges
Microscopic Pathology:
- reticular variant - uniformly distributed cells within intricate network of capillaries
- cellular variant - cells clustered forming groups separated by capillaries
- stromal cells
- irregular hyperchromatic nuclei
- eosinophilic, variably lipid rich, ill defined cytoplasm
- abundant glycogen
- mitoses absent or infrequent
- extramedullary erythropoiesis may be seen
Immunoreactivity:
- + vimentin,
- + neuron specific enolase
- +/- GFAP
- epithelial membrane antigen
Genetics:
- inactivation of the vHL tumor suppressor gene
- some sporadic cases
- von Hippel-Lindau disease
Clinical manifestations:
1) may cause symptoms by compression of local neural structures
a) symptoms depend on location; i.e cerebellar ataxia, motor weakness, sensory deficits
b) pain may be a symptom
2) bleeding may occur
3) paraneoplastic syndrome may develop
Radiology:
1) magnetic resonance imaging (MRI) with gadolinium contrast
a) enhancing mass frequently associated with a cyst
b) smaller hemangiomas (< 10 mm) may be isointense on T1-weighted images & hyperintense on T2-weighted images
2) angiography in conjunction with CT for patients who are not candidates for MRI
Complications:
1) compression of local neural structures
2) spinal cord edema
2) acute hemorrhage can be catastrophic
Differential diagnosis:
1) astrocytoma
2) ganglioneuroma
3) metastatic tumor
Management:
1) tumors best managed by multidisciplinary team including
a) neuroradiologist, radiation oncologist
b) neurosurgeon
c) neurooncologist
2) preoperative angiogram
3) embolization of feeding arteries may be indicated
- microspheres, ethanol
4) intra-operative microscopic microdissection
5) stereotactic radiosurgery (gamma-knife radiosurgery)
6) conventional radiation therapy
- recurrence-free survival rates at 5,10 & 15 years are 76, 52 & 42%.
Interactions
disease interactions
General
central nervous system (CNS) neoplasm
vascular tissue neoplasm; angioma
References
- WHO International Histological Classification of Tumors.
Histological Typing of Tumours of the Central Nervous System.
Kleihues et al. Springer-Verlag 2nd ed. 1993
- UpToDate version 12.3