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#redirect [[Vascular_tumours#Kaposi_sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Kaposi_sarcoma_high_mag.jpg
| Width      =
| Caption    = Kaposi sarcoma. [[H&E stain]].
| Micro      = vascular lesion (abundant [[RBC]]s), +/-"promontory sign", +/-spindle cells with minimal nuclear atypia, [[RBC extravasation]], +/-intracytoplasmic [[hyaline globules]], +/-hemosiderin deposits, +/-plasma cells
| Subtypes  = classic, endemic, immunosuppression-associated ''or'' transplant-associated, AIDS-associated
| LMDDx      = [[angiosarcoma]], [[Masson's hemangioma]] (''intravascular papillary endothelial hyperplasia''), benign lymphangioendothelioma
| Stains    =
| IHC        = [[HHV-8]] +ve, CD31 +ve, CD34 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = red or brown patches, plaques or nodules
| Grossing  =
| Site      = [[skin]], others
| Assdx      = +/-[[HIV]] infection ''or'' immunoincompentence
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = dependent on subtype
| Other      =
| ClinDDx    =
}}
'''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.
 
==Gross==
*Skin: red or brown patches, plaques or nodules.
 
<gallery>
Image:Kaposis_sarcoma_01.jpg | KS. (WC)
</gallery>
==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>
*[[Cavernous hemangioma]].<ref name=pmid24187557>{{cite journal |author=Onak Kandemir N, Barut F, Doğan Gün B, Solak Tekin N, Hallaç Keser S, Oğuz Özdamar S |title=Cavernous hemangioma-like kaposi sarcoma: histomorphologic features and differential diagnosis |journal=Case Rep Med |volume=2013 |issue= |pages=959812 |year=2013 |pmid=24187557 |pmc=3800618 |doi=10.1155/2013/959812 |url=}}</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.
*[[Hale's colloidal iron stain]] +ve.<ref name=pmid17210517>{{cite journal |author=Wada DA, Perkins SL, Tripp S, Coffin CM, Florell SR |title=Human herpesvirus 8 and iron staining are useful in differentiating Kaposi sarcoma from interstitial granuloma annulare |journal=Am. J. Clin. Pathol. |volume=127 |issue=2 |pages=263–70 |year=2007 |month=February |pmid=17210517 |doi=10.1309/GMH9CENH4909AWVB |url=}}</ref>
 
==IHC==
Features:<ref name=pmid24187557/>
*CD31 +ve.
*CD34 +ve.
*[[HHV-8]] +ve.
*D2-40 +ve.
*Ki-67 >10%.<ref name=pmid17210517>{{cite journal |author=Wada DA, Perkins SL, Tripp S, Coffin CM, Florell SR |title=Human herpesvirus 8 and iron staining are useful in differentiating Kaposi sarcoma from interstitial granuloma annulare |journal=Am. J. Clin. Pathol. |volume=127 |issue=2 |pages=263–70 |year=2007 |month=February |pmid=17210517 |doi=10.1309/GMH9CENH4909AWVB |url=}}</ref>
*ERG +ve. <ref name=pmid21317715>{{cite journal |author=Miettinen M1, Wang ZF, Paetau A, Tan SH, Dobi A, Srivastava S, Sesterhenn I. |title=ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. |journal=Am J Surg Pathol. 2011 Mar;35(3):432-41. |volume=35|issue=3 |pages=432-41 |year=2011 |month=march |pmid=21317715 |doi: 10.1097/PAS.0b013e318206b67b|url=http://www.ncbi.nlm.nih.gov/pubmed/21317715}}</ref>
==See also==
*[[Vascular tumours]].
*[[Angiosarcoma]].
 
==References==
{{Reflist|2}}
 
[[Category:Vascular tumours]]
[[Category:Diagnosis]]
Michael
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