Difference between revisions of "Hemangioblastoma"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Cerebellar_hemangioblastoma_high_mag.jpg
| Image      = Hemangioblastoma Histology HE.jpg
| Width      =
| Width      =
| Caption    = Cerebellar hemangioblastoma.
| Caption    = Cerebellar hemangioblastoma.
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| Micro      = vascular tumour with large polygonal stromal cells with hyperchromatic nuclei and vacuolar cytoplasm
| Micro      = vascular tumour with large polygonal stromal cells with hyperchromatic nuclei and vacuolar cytoplasm
| Subtypes  =
| Subtypes  =
| LMDDx      = metastatic [[cell cell renal cell carcinoma]]
| LMDDx      = metastatic [[clear cell renal cell carcinoma]]
| Stains    =
| Stains    =
| IHC        = alpha-inhibin +ve, NSE +ve, EMA -ve
| IHC        = alpha-inhibin +ve, NSE +ve, EMA -ve
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==General==
==General==
*Usually ''cerebellar''.
*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]].
*Associated with [[von Hippel-Lindau syndrome]].
*WHO grade I.<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>
*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==
==Microscopic==
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**Hyperchromatic nuclei.
**Hyperchromatic nuclei.
**Vacuolar cytoplasm.
**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:
DDx:
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Image:Cerebellar_hemangioblastoma_high_mag.jpg | Hemangioblastoma - high mag. (WC)
Image:Cerebellar_hemangioblastoma_high_mag.jpg | Hemangioblastoma - high mag. (WC)
Image:Hemangioblastoma_-_nse_-_intermed_mag.jpg | Hemangioblastoma - NSE - intermed. 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>
</gallery>
www:
www:
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*NSE +ve (nucleus + cytoplasm).
*NSE +ve (nucleus + cytoplasm).
**RCC typically -ve.
**RCC typically -ve.
*CK -ve.


==See also==
==See also==
*[[Von Hippel-Lindau disease]].
*[[Von Hippel-Lindau disease]].
*[[Neuropathology tumours]].
*[[Neuropathology tumours]].
*[[Hemangioblastoma of the kidney]].


==References==
==References==

Latest revision as of 13:57, 19 March 2025

Hemangioblastoma
Diagnosis in short
Template:Px
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

  • Cerebellar hemangioblastoma intermed mag.jpg

    Hemangioblastoma - intermed. mag. (WC)

  • Cerebellar hemangioblastoma high mag.jpg

    Hemangioblastoma - high mag. (WC)

  • Hemangioblastoma - nse - intermed mag.jpg

    Hemangioblastoma - NSE - intermed. mag. (WC)

  • Hemangioblastoma - nse - high mag.jpg

    Hemangioblastoma - NSE - high mag. (WC)

  • Hemangioblastoma Histology CD31.jpg

    Hemangioblastoma - CD31. (WC/marvin101)

  • Hippel Lindau.gif

    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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