Difference between revisions of "Kaposi sarcoma"

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#redirect [[Vascular_tumours#Kaposi_sarcoma]]
'''Kaposi sarcoma''', abbreviated '''KS''', is an uncommon [[vascular tumour]] that is often associated with [[HIV]]/AIDS.


==General==
*Caused by [[Human herpesvirus-8]] (HHV-8).
*In the North American context, it is often associated with immunodeficiency, e.g. [[HIV]]/AIDS.
Interesting note:
*It has been said that KS is not really a sarcoma.<ref name=pmid14521707>{{Cite journal  | last1 = Pérez | first1 = A. | last2 = Sánchez | first2 = JL. | last3 = Almodóvar | first3 = PI. | title = Kaposi's sarcoma is not a neoplasm let alone a sarcoma. | journal = Int J Dermatol | volume = 42 | issue = 10 | pages = 844-5 | month = Oct | year = 2003 | doi =  | PMID = 14521707 }}</ref>
===Stages===
It is seen in different stages:<ref>URL: [http://www.histopathology-india.net/KS.htm http://www.histopathology-india.net/KS.htm]. Accessed on: 31 January 2010.</ref><ref>URL: [http://emedicine.medscape.com/article/1083998-clinical#a0217 http://emedicine.medscape.com/article/1083998-clinical#a0217]. Accessed on: 17 November 2011.</ref>
#Patch stage.
#Plaque stage.
#Nodular stage.
#Exophytic stage.
#Infiltrative stage.
#Lymphadenopathic stage.
Note:
*The first three are the classic ones.
===Type or form===
Classically divided into four types:<ref name=pmid18219366>{{Cite journal  | last1 = Szajerka | first1 = T. | last2 = Jablecki | first2 = J. | title = Kaposi's sarcoma revisited. | journal = AIDS Rev | volume = 9 | issue = 4 | pages = 230-6 | month =  | year =  | doi =  | PMID = 18219366 }}</ref><ref name=pmid17691428>{{Cite journal  | last1 = Morand | first1 = JJ. | last2 = Lightburn | first2 = E. | last3 = Simon | first3 = F. | last4 = Patte | first4 = JH. | title = [Update on Kaposi's sarcoma]. | journal = Med Trop (Mars) | volume = 67 | issue = 2 | pages = 123-30 | month = Apr | year = 2007 | doi =  | PMID = 17691428 }}</ref><ref name=pmid10749966>{{Cite journal  | last1 = Antman | first1 = K. | last2 = Chang | first2 = Y. | title = Kaposi's sarcoma. | journal = N Engl J Med | volume = 342 | issue = 14 | pages = 1027-38 | month = Apr | year = 2000 | doi = 10.1056/NEJM200004063421407 | PMID = 10749966 | URL = http://www.nejm.org/doi/full/10.1056/NEJM200004063421407 }}</ref>
*Classic = old men Mediterranean or Ashkenazi Jew.
*Endemic = African infants and young males.
*Immunosuppression-associated or transplant-associated - iatrogenic.
*AIDS-associated.
==Microscopic==
Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>
*Vascular lesion (abundant [[RBC]]s) with:
**+/-"Promontory sign" - small vessel protruding into an abnormal vascular space.<ref name=pmid19318797>{{cite journal |author=Lazova R, McNiff JM, Glusac EJ, Godic A |title=Promontory sign--present in patch and plaque stage of angiosarcoma! |journal=Am J Dermatopathol |volume=31 |issue=2 |pages=132–6 |year=2009 |month=April |pmid=19318797 |doi=10.1097/DAD.0b013e3181951045 |url=}}</ref>
***Not pathognomonic for KS.<ref name=pmid20577080>{{cite journal |author=Fernandez-Flores A, Rodriguez R |title=Promontory Sign in a Reactive Benign Vascular Proliferation |journal=Am J Dermatopathol |volume= |issue= |pages= |year=2010 |month=June |pmid=20577080 |doi=10.1097/DAD.0b013e3181cf0ae5 |url=}}</ref>
**+/-Spindle cells with minimal nuclear atypia.
**[[RBC extravasation]] - very useful - '''important feature'''.<ref name=pmid2229644>{{Cite journal  | last1 = Kato | first1 = H. | last2 = Hamada | first2 = T. | last3 = Tsuji | first3 = T. | last4 = Baba | first4 = T. | last5 = Seki | first5 = J. | last6 = Kobayashi | first6 = Y. | title = Kaposi's sarcoma: a light and electron microscopic study. | journal = J Dermatol | volume = 17 | issue = 7 | pages = 414-22 | month = Jul | year = 1990 | doi =  | PMID = 2229644 }}</ref>
*+/-Intracytoplasmic [[hyaline globules]] - uncommon - one usu. needs to search for 'em.<ref name=pmid7528163>{{cite journal |author=del Rosario AD, Bui HX, Singh J, Ginsburg R, Ross JS |title=Intracytoplasmic eosinophilic hyaline globules in cartilaginous neoplasms: a surgical, pathological, ultrastructural, and electron probe x-ray microanalytic study |journal=Hum. Pathol. |volume=25 |issue=12 |pages=1283–9 |year=1994 |month=December |pmid=7528163 |doi= |url=}}</ref>
**Pale pink globs (that are paler than RBCs) - '''important feature'''.
*+/-Hemosiderin deposits.
*+/-Plasma cells.<ref name=pmid17912148>{{Cite journal  | last1 = Douglas | first1 = JL. | last2 = Gustin | first2 = JK. | last3 = Dezube | first3 = B. | last4 = Pantanowitz | first4 = JL. | last5 = Moses | first5 = AV. | title = Kaposi's sarcoma: a model of both malignancy and chronic inflammation. | journal = Panminerva Med | volume = 49 | issue = 3 | pages = 119-38 | month = Sep | year = 2007 | doi =  | PMID = 17912148 }}</ref>
Notes:
*[[Hyaline globules]] have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/>
DDx:
*[[Angiosarcoma]] - have many mitoses, nuclear atypia, RBC extravasation not common.
*[[Masson's hemangioma]] - [[AKA]] ''intravascular papillary endothelial hyperplasia''.
*Benign lymphangioendothelioma.<ref>{{Cite journal  | last1 = Guillou | first1 = L. | last2 = Fletcher | first2 = CD. | title = Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series. | journal = Am J Surg Pathol | volume = 24 | issue = 8 | pages = 1047-57 | month = Aug | year = 2000 | doi =  | PMID = 10935645 }}</ref>
**Histologically very similar.<ref>URL: [http://path.upmc.edu/cases/case134/dx.html http://path.upmc.edu/cases/case134/dx.html]. Accessed on: 5 January 2012.</ref>
===Images===
<gallery>
Image:Kaposi_sarcoma_high_mag.jpg | Kaposi sacoma - high mag. (WC)
Image:Kaposi_sarcoma_low_intermed_mag.jpg | Kaposi sarcoma - intermed. mag. (WC)
</gallery>
www:
*[http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].
==Stains==
*PAS +ve -- hyaline globules.
==IHC==
*CD31 +ve.
*CD34 +ve.
*[[HHV-8]] +ve.
==See also==
*[[Vascular tumours]].
*[[Angiosarcoma]].
==References==
{{Reflist|2}}
[[Category:Vascular tumours]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Revision as of 01:08, 15 October 2013

Kaposi sarcoma, abbreviated KS, is an uncommon vascular tumour that is often associated with HIV/AIDS.

General

  • Caused by Human herpesvirus-8 (HHV-8).
  • In the North American context, it is often associated with immunodeficiency, e.g. HIV/AIDS.

Interesting note:

  • It has been said that KS is not really a sarcoma.[1]

Stages

It is seen in different stages:[2][3]

  1. Patch stage.
  2. Plaque stage.
  3. Nodular stage.
  4. Exophytic stage.
  5. Infiltrative stage.
  6. Lymphadenopathic stage.

Note:

  • The first three are the classic ones.

Type or form

Classically divided into four types:[4][5][6]

  • Classic = old men Mediterranean or Ashkenazi Jew.
  • Endemic = African infants and young males.
  • Immunosuppression-associated or transplant-associated - iatrogenic.
  • AIDS-associated.

Microscopic

Features:[7]

  • Vascular lesion (abundant RBCs) with:
    • +/-"Promontory sign" - small vessel protruding into an abnormal vascular space.[8]
      • Not pathognomonic for KS.[9]
    • +/-Spindle cells with minimal nuclear atypia.
    • RBC extravasation - very useful - important feature.[10]
  • +/-Intracytoplasmic hyaline globules - uncommon - one usu. needs to search for 'em.[11]
    • Pale pink globs (that are paler than RBCs) - important feature.
  • +/-Hemosiderin deposits.
  • +/-Plasma cells.[12]

Notes:

DDx:

  • Angiosarcoma - have many mitoses, nuclear atypia, RBC extravasation not common.
  • Masson's hemangioma - AKA intravascular papillary endothelial hyperplasia.
  • Benign lymphangioendothelioma.[13]
    • Histologically very similar.[14]

Images

www:

Stains

  • PAS +ve -- hyaline globules.

IHC

  • CD31 +ve.
  • CD34 +ve.
  • HHV-8 +ve.

See also

References

  1. Pérez, A.; Sánchez, JL.; Almodóvar, PI. (Oct 2003). "Kaposi's sarcoma is not a neoplasm let alone a sarcoma.". Int J Dermatol 42 (10): 844-5. PMID 14521707.
  2. URL: http://www.histopathology-india.net/KS.htm. Accessed on: 31 January 2010.
  3. URL: http://emedicine.medscape.com/article/1083998-clinical#a0217. Accessed on: 17 November 2011.
  4. Szajerka, T.; Jablecki, J.. "Kaposi's sarcoma revisited.". AIDS Rev 9 (4): 230-6. PMID 18219366.
  5. Morand, JJ.; Lightburn, E.; Simon, F.; Patte, JH. (Apr 2007). "[Update on Kaposi's sarcoma].". Med Trop (Mars) 67 (2): 123-30. PMID 17691428.
  6. Antman, K.; Chang, Y. (Apr 2000). "Kaposi's sarcoma.". N Engl J Med 342 (14): 1027-38. doi:10.1056/NEJM200004063421407. PMID 10749966.
  7. Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 23. ISBN 978-1416002741.
  8. Lazova R, McNiff JM, Glusac EJ, Godic A (April 2009). "Promontory sign--present in patch and plaque stage of angiosarcoma!". Am J Dermatopathol 31 (2): 132–6. doi:10.1097/DAD.0b013e3181951045. PMID 19318797.
  9. Fernandez-Flores A, Rodriguez R (June 2010). "Promontory Sign in a Reactive Benign Vascular Proliferation". Am J Dermatopathol. doi:10.1097/DAD.0b013e3181cf0ae5. PMID 20577080.
  10. Kato, H.; Hamada, T.; Tsuji, T.; Baba, T.; Seki, J.; Kobayashi, Y. (Jul 1990). "Kaposi's sarcoma: a light and electron microscopic study.". J Dermatol 17 (7): 414-22. PMID 2229644.
  11. 11.0 11.1 del Rosario AD, Bui HX, Singh J, Ginsburg R, Ross JS (December 1994). "Intracytoplasmic eosinophilic hyaline globules in cartilaginous neoplasms: a surgical, pathological, ultrastructural, and electron probe x-ray microanalytic study". Hum. Pathol. 25 (12): 1283–9. PMID 7528163.
  12. Douglas, JL.; Gustin, JK.; Dezube, B.; Pantanowitz, JL.; Moses, AV. (Sep 2007). "Kaposi's sarcoma: a model of both malignancy and chronic inflammation.". Panminerva Med 49 (3): 119-38. PMID 17912148.
  13. Guillou, L.; Fletcher, CD. (Aug 2000). "Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.". Am J Surg Pathol 24 (8): 1047-57. PMID 10935645.
  14. URL: http://path.upmc.edu/cases/case134/dx.html. Accessed on: 5 January 2012.