Hemangioblastoma

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Hemangioblastoma
Diagnosis in short

Cerebellar hemangioblastoma.

LM vascular tumour with large polygonal stromal cells with hyperchromatic nuclei and vacuolar cytoplasm
LM DDx metastatic clear cell renal cell carcinoma
IHC alpha-inhibin +ve, NSE +ve, EMA -ve
Site brain - usu. cerebellum

Syndromes von Hippel-Lindau disease

Prognosis good (WHO grade I)

Hemangioblastoma is a low grade brain tumour tumour typically found the cerebellum.

General

  • Usually cerebellar.
    • occassionally brainstem or spinal cord. Supratentorial tumors are exceptionally rare.
  • Typically in adults.[1]
  • Associated with von Hippel-Lindau syndrome.
  • Symptomatic when CSF flow is impaired.
  • WHO grade I (ICD-O: 9161/1).[2]

Macroscopy

  • Red to yellow nodules, highly vascularized.
  • 75% are cystic tumors.
    • Peripheral solid portion.


Microscopic

Features:[3]

  • Vascular.
  • Polygonal stromal cells with:
    • Hyperchromatic nuclei.
    • Vacuolar cytoplasm.
  • Occassionally extensive sclerosis.

Note:

  • Based on the stromal content, some classify the tumors as "cellular" and "reticular".[4]


DDx:

Images

www:

IHC

Features:[5]

  • Alpha-inhibin +ve (cytoplasm).
  • EMA -ve.
    • RCC typically +ve.
  • NSE +ve (nucleus + cytoplasm).
    • RCC typically -ve.
  • CK -ve.

See also

References

  1. Kassardjian, CD.; Macdonald, RL.; Munoz, DG. (Jul 2014). "Hemangioblastomas in the elderly: epidemiology and clinical characteristics.". J Clin Neurosci 21 (7): 1205-8. doi:10.1016/j.jocn.2013.10.023. PMID 24629394.
  2. URL: http://www.expertconsultbook.com/expertconsult/ob/book.do?method=display&type=bookPage&decorator=none&eid=4-u1.0-B978-1-4160-4580-9..00019-8--sc0155&isbn=978-1-4160-4580-9. Accessed on: 9 December 2010.
  3. URL: http://emedicine.medscape.com/article/340994-media. Accessed on: 23 June 2010.
  4. Hasselblatt, M.; Jeibmann, A.; Gerss, J.; Behrens, C.; Rama, B.; Wassmann, H.; Paulus, W. (Dec 2005). "Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases.". Neuropathol Appl Neurobiol 31 (6): 618-22. doi:10.1111/j.1365-2990.2005.00669.x. PMID 16281910.
  5. URL: http://www.nature.com/modpathol/journal/v18/n6/full/3800351a.html. Accessed on: 9 December 2010.