Difference between revisions of "Hemangioblastoma"

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#redirect [[Neuropathology tumours#Hemangioblastoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Hemangioblastoma Histology HE.jpg
| Width      =
| Caption    = Cerebellar hemangioblastoma.
| Synonyms  =
| Micro      = vascular tumour with large polygonal stromal cells with hyperchromatic nuclei and vacuolar cytoplasm
| Subtypes  =
| LMDDx      = metastatic [[clear cell renal cell carcinoma]]
| Stains    =
| IHC        = alpha-inhibin +ve, NSE +ve, EMA -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = brain - usu. [[cerebellum]]
| Assdx      =
| Syndromes  = [[von Hippel-Lindau disease]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good (WHO grade I)
| Other      =
| ClinDDx    =
| Tx        =
}}
'''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.<ref>{{Cite journal  | last1 = Kassardjian | first1 = CD. | last2 = Macdonald | first2 = RL. | last3 = Munoz | first3 = DG. | title = Hemangioblastomas in the elderly: epidemiology and clinical characteristics. | journal = J Clin Neurosci | volume = 21 | issue = 7 | pages = 1205-8 | month = Jul | year = 2014 | doi = 10.1016/j.jocn.2013.10.023 | PMID = 24629394 }}</ref>
*Associated with [[von Hippel-Lindau syndrome]].
*Symptomatic when CSF flow is impaired.
*WHO grade I (ICD-O: 9161/1).<ref>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 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.</ref>
 
==Macroscopy==
*Red to yellow nodules, highly vascularized.
*75% are cystic tumors.
**Peripheral solid portion.
 
 
==Microscopic==
Features:<ref>URL: [http://emedicine.medscape.com/article/340994-media http://emedicine.medscape.com/article/340994-media]. Accessed on: 23 June 2010.</ref>
*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".<ref>{{Cite journal  | last1 = Hasselblatt | first1 = M. | last2 = Jeibmann | first2 = A. | last3 = Gerss | first3 = J. | last4 = Behrens | first4 = C. | last5 = Rama | first5 = B. | last6 = Wassmann | first6 = H. | last7 = Paulus | first7 = W. | title = Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. | journal = Neuropathol Appl Neurobiol | volume = 31 | issue = 6 | pages = 618-22 | month = Dec | year = 2005 | doi = 10.1111/j.1365-2990.2005.00669.x | PMID = 16281910 }}</ref>
 
 
DDx:
*Metastatic [[clear cell renal cell carcinoma]].
 
===Images===
<gallery>
Image:Cerebellar_hemangioblastoma_intermed_mag.jpg | Hemangioblastoma - intermed. mag. (WC)
Image:Cerebellar_hemangioblastoma_high_mag.jpg | Hemangioblastoma - high mag. (WC)
Image:Hemangioblastoma_-_nse_-_intermed_mag.jpg | Hemangioblastoma - NSE - intermed. mag. (WC)
File:Hemangioblastoma - nse - high mag.jpg | Hemangioblastoma - NSE - high mag. (WC)
File:Hemangioblastoma Histology CD31.jpg | Hemangioblastoma - CD31. (WC/marvin101)
File:Hippel Lindau.gif | Hemangioblastomas in Hippel-Lindau disease (PlosONE).
</gallery>
www:
*[http://path.upmc.edu/cases/case342.html Hemangioblastoma - several images (upmc.edu)].
 
==IHC==
Features:<ref>URL: [http://www.nature.com/modpathol/journal/v18/n6/full/3800351a.html http://www.nature.com/modpathol/journal/v18/n6/full/3800351a.html]. Accessed on: 9 December 2010.</ref>
*Alpha-inhibin +ve (cytoplasm).
*EMA -ve.
**RCC typically +ve.
*NSE +ve (nucleus + cytoplasm).
**RCC typically -ve.
*CK -ve.
 
==See also==
*[[Von Hippel-Lindau disease]].
*[[Neuropathology tumours]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Neuropathology tumours]]

Latest revision as of 07:48, 22 May 2015

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

  • Hemangioblastoma - intermed. mag. (WC)

  • Hemangioblastoma - high mag. (WC)

  • Hemangioblastoma - NSE - intermed. mag. (WC)

  • Hemangioblastoma - NSE - high mag. (WC)

  • Hemangioblastoma - CD31. (WC/marvin101)

  • Hemangioblastomas in Hippel-Lindau disease (PlosONE).

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.
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