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[[Image:Capillary_hemangioma_-_very_high_mag.jpg|thumb|right|[[Micrograph]] showing a [[capillary hemangioma]], a common type of vascular tumour. [[H&E stain]].]]
This article covers [[soft tissue lesions|soft tissue]] '''vascular tumours'''.  Vascular malformations are covered in the ''[[vascular malformations]]'' article.
This article covers [[soft tissue lesions|soft tissue]] '''vascular tumours'''.  Vascular malformations are covered in the ''[[vascular malformations]]'' article.


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=Distinct entities=
=Distinct entities=
==Hemangioma==
==Hemangioma==
===General===
{{Main|Hemangioma}}
*May be found in the liver.
{{Main|Liver hemangioma}}
**Classically subcapsular.
***May rupture and be life-threatening.<ref>{{Cite journal  | last1 = Vokaer | first1 = B. | last2 = Kothonidis | first2 = K. | last3 = Delatte | first3 = P. | last4 = De Cooman | first4 = S. | last5 = Pector | first5 = JC. | last6 = Liberale | first6 = G. | title = Should ruptured liver haemangioma be treated by surgery or by conservative means? A case report. | journal = Acta Chir Belg | volume = 108 | issue = 6 | pages = 761-4 | month =  | year =  | doi =  | PMID = 19241936 }}</ref>
 
Hemangiomas to remember - if you're only going remember a few:
*''Glomeruloid'', ''infantile'', ''caverous'', ''capillary'', ''arteriovenous'', ''venous'' and ''intramuscular''.
 
====Childhood====
Common childhood hemangiomas:<ref name=pmid10410855>{{cite journal |author=Prieto VG, Shea CR |title=Selected cutaneous vascular neoplasms. A review |journal=Dermatol Clin |volume=17 |issue=3 |pages=507–20, viii |year=1999 |month=July |pmid=10410855 |doi= |url=}}</ref>
*Tufted - small clusters of blood vessels.
*Microvenular hemangioma.
*Glomeruloid hemangioma - associated with [[POEMS syndrome]], [[Castleman disease]].<ref name=pmid16860182>{{cite journal |author=Uthup S, Balachandran K, Ammal VA, ''et al.'' |title=Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma |journal=Am. J. Kidney Dis. |volume=48 |issue=2 |pages=e17–24 |year=2006 |month=August |pmid=16860182 |doi=10.1053/j.ajkd.2006.04.089 |url=}}</ref><ref name=Ref_WMSP618>{{Ref WMSP|618}}</ref>
*Epithelioid hemangioma.
*Targetoid hemosideric hemangioma.
*Infantile hemangioma (AKA juvenile hemangioma<ref name=pmid10665907>{{Cite journal  | last1 = North | first1 = PE. | last2 = Waner | first2 = M. | last3 = Mizeracki | first3 = A. | last4 = Mihm | first4 = MC. | title = GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. | journal = Hum Pathol | volume = 31 | issue = 1 | pages = 11-22 | month = Jan | year = 2000 | doi =  | PMID = 10665907 }}</ref>) - these tumours are ''GLUT-1'' +ve.  They tumours grow and then spontaneously regress.<ref name=pmid15143338>{{Cite journal  | last1 = Dadras | first1 = SS. | last2 = North | first2 = PE. | last3 = Bertoncini | first3 = J. | last4 = Mihm | first4 = MC. | last5 = Detmar | first5 = M. | title = Infantile hemangiomas are arrested in an early developmental vascular differentiation state. | journal = Mod Pathol | volume = 17 | issue = 9 | pages = 1068-79 | month = Sep | year = 2004 | doi = 10.1038/modpathol.3800153 | PMID = 15143338 }}</ref>
 
====Soft tissue====
Several types are seen in soft tissue:<ref>{{Ref WMSP|602}}</ref>
*Capillary.
*Cavernous.
*Arteriovenous.
*Venous.
*Intramuscular.
*Synovial.
 
===Microscopic===
Features:
*Channels lined by endothelium containing [[RBC]]s.
 
DDx:
*[[Lymphangioma]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_intermed_mag.jpg Capillary hemangioma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_very_high_mag.jpg Capillary hemangioma - very high mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case277.html Diffuse hemangioma - several images (upmc.edu)].
 
===IHC===
*CD31 +ve.
*D2-40 -ve.<ref name=pmid11950918/>
 
''Juvenile hemangioma'':<ref name=pmid10665907/>
*GLUT-1 +ve.


==Lymphangioma==
==Lymphangioma==
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===IHC===
===IHC===
Features:<ref name=pmid19098468>{{Cite journal  | last1 = Kalof | first1 = AN. | last2 = Cooper | first2 = K. | title = D2-40 immunohistochemistry--so far! | journal = Adv Anat Pathol | volume = 16 | issue = 1 | pages = 62-4 | month = Jan | year = 2009 | doi = 10.1097/PAP.0b013e3181915e94 | PMID = 19098468 }}</ref><ref name=pmid11950918/>
Features:<ref name=pmid19098468>{{Cite journal  | last1 = Kalof | first1 = AN. | last2 = Cooper | first2 = K. | title = D2-40 immunohistochemistry--so far! | journal = Adv Anat Pathol | volume = 16 | issue = 1 | pages = 62-4 | month = Jan | year = 2009 | doi = 10.1097/PAP.0b013e3181915e94 | PMID = 19098468 }}</ref><ref name=pmid11950918>{{Cite journal  | last1 = Kahn | first1 = HJ. | last2 = Bailey | first2 = D. | last3 = Marks | first3 = A. | title = Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas. | journal = Mod Pathol | volume = 15 | issue = 4 | pages = 434-40 | month = Apr | year = 2002 | doi = 10.1038/modpathol.3880543 | PMID = 11950918 }}</ref>
*D2-40 +ve.
*D2-40 +ve.


==Kaposi sarcoma==
==Kaposi sarcoma==
*Abbreviated ''KS''.
{{Main|Kaposi sarcoma}}
===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:
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
*[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.


==Masson hemangioma==
==Masson hemangioma==
*[[AKA]] ''intravascular papillary endothelial hyperplasia''.<ref name=pmid15865098>{{cite journal |author=Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma |journal=Anticancer Res. |volume=25 |issue=2B |pages=1409–12 |year=2005 |pmid=15865098 |doi= |url=}}</ref>  
*[[AKA]] ''intravascular papillary endothelial hyperplasia''.<ref name=pmid15865098>{{cite journal |author=Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma |journal=Anticancer Res. |volume=25 |issue=2B |pages=1409–12 |year=2005 |pmid=15865098 |doi= |url=}}</ref>  
*[[AKA]] ''Masson tumor''.<ref>URL: [http://path.upmc.edu/cases/case544/dx.html http://path.upmc.edu/cases/case544/dx.html]. Accessed on: 25 January 2012.</ref>
*[[AKA]] ''Masson tumor''.<ref>URL: [http://path.upmc.edu/cases/case544/dx.html http://path.upmc.edu/cases/case544/dx.html]. Accessed on: 25 January 2012.</ref>
===General===
{{Main|intravascular papillary endothelial hyperplasia}}
*Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
*Histomorphologically may be confused with low-grade [[angiosarcoma]] or other soft tissue sarcomas.<ref name=pmid15865098/>
===Microscopic===
Features:
*Well-circumscribed - '''key (low power) feature'''.
*Abundant small vascular channels with benign endothelium.
*+/-Papillary formation with a fibrotic core covered by benign endothelium.<ref>URL: [http://path.upmc.edu/cases/case544.html http://path.upmc.edu/cases/case544.html]. Accessed on: 25 January 2012.</ref>
 
Notes:
*Looks like [[Kaposi sarcoma]] at high power.
 
Images:
*[http://path.upmc.edu/cases/case544.html Masson hemangioma - several images (upmc.edu)].


==Angiosarcoma==
==Angiosarcoma==
===General===
{{Main|Angiosarcoma}}
*Malignant tumour - with a horrible prognosis.<ref name=pmid20537949>{{cite journal |author=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol |volume= |issue= |pages= |year=2010 |month=May |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Classically on the scalp or head & neck.
 
Epidemiology:
*May arise secondary to therapeutic radiation or chronic lymphoedema related to breast carcinoma.
*Liver angiosarcomas are assoc. with vinyl chloride exposure.<ref>{{Ref PCPBoD8|212}}</ref>
 
===Microscopic===
Features:
*Very many small capillaries of irregular shape lined with:
**Pleomorphic nuclei.
***May have hobnail morphology.
*Mitoses.
*Cytoplasmic vacuoles.
**Cells trying to form lumina - embryologic.
 
Notes:
*Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble ''[[melanoma]]'' or ''[[hepatocellular carcinoma]]''.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_low_mag.jpg Epithelioid angiosarcoma - very low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_intermed_mag.jpg Epithelioid angiosarcoma - intermed mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_high_mag.jpg Epithelioid angiosarcoma - very high mag. (WC)].
 
===IHC===
*CD34 +ve.
*CD31 +ve.
*FLI-1 +ve.<ref name=pmid15001993>{{Cite journal  | last1 = Rossi | first1 = S. | last2 = Orvieto | first2 = E. | last3 = Furlanetto | first3 = A. | last4 = Laurino | first4 = L. | last5 = Ninfo | first5 = V. | last6 = Dei Tos | first6 = AP. | title = Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. | journal = Mod Pathol | volume = 17 | issue = 5 | pages = 547-52 | month = May | year = 2004 | doi = 10.1038/modpathol.3800065 | PMID = 15001993 }}</ref>
*D2-40 +ve/-ve.<ref name=pmid11950918>{{Cite journal  | last1 = Kahn | first1 = HJ. | last2 = Bailey | first2 = D. | last3 = Marks | first3 = A. | title = Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas. | journal = Mod Pathol | volume = 15 | issue = 4 | pages = 434-40 | month = Apr | year = 2002 | doi = 10.1038/modpathol.3880543 | PMID = 11950918 | URL = http://www.nature.com/modpathol/journal/v15/n4/full/3880543a.html }}</ref>
*HHV-8 -ve.


==Kaposiform hemangioendothelioma==
==Kaposiform hemangioendothelioma==
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==Epithelioid hemangioendothelioma==
==Epithelioid hemangioendothelioma==
*Should '''not''' be confused with ''[[epithelioid hemangioma]]''.
{{Main|Epithelioid hemangioendothelioma}}
===General===
*Malignant.<ref name=Ref_WMSP603>{{Ref WMSP|603}}</ref>
*Adults - wide age range.
*Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/>


===Microscopic===
==Retiform hemangioendothelioma==
Features:<ref name=pmid20165548>{{Cite journal  | last1 = Gupta | first1 = R. | last2 = Mathur | first2 = SR. | last3 = Gupta | first3 = SD. | last4 = Durgapal | first4 = P. | last5 = Iyer | first5 = VK. | last6 = Das | first6 = CJ. | last7 = Shalimar | first7 = SK. | last8 = Acharya | first8 = . | title = Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology. | journal = Cytojournal | volume = 6 | issue =  | pages = 25 | month =  | year = 2010 | doi = 10.4103/1742-6413.58951 | PMID = 20165548 | URL = http://www.biomedsearch.com/nih/Hepatic-epithelioid-hemangioendothelioma-diagnostic-pitfall/20165548.html }}</ref>
{{Main|Retiform hemangioendothelioma}}
*Large epithelioid perivascular cells with:
**Abundant pale eosinophilic cytoplasm.
**Cytoplasmic vacuolation (some cells) - [[AKA]] "blister cells" - '''key feature'''.
***May form lumen and have RBC within.
**Vesicular nucleus with prominent nucleolus in some cells.
*Tuft-like projections into capillaries.
*Tumour cells may be in well-circumscribed paucicellular nodules ''or'' more cellular poorly formed aggregates.


DDx:
==Intimal sarcoma==
*[[Angiosarcoma]], epithelioid.
{{Main|Intimal sarcoma}}
*[[Hemangioma]].
 
Images:
*[http://en.wikipedia.org/wiki/File:Epithelioid_hemangioendothelioma_EHE.Image5.jpg Epithelioid hemangioendothelioma (WP)].
*[http://www.flickr.com/photos/pulmonary_pathology/5600772691/in/photostream/ Epithelioid hemangioendothelioma - low mag. (flickr.com/Rosen)].
*[http://www.flickr.com/photos/pulmonary_pathology/5601354962/ Epithelioid hemangioendothelioma - high mag. (flickr.com/Rosen)].
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802170854495 Epithelioid hemangioendothelioma (surgicalpathologyatlas.com)].
 
===IHC===
Features:<ref name=pmid20165548>{{Cite journal  | last1 = Gupta | first1 = R. | last2 = Mathur | first2 = SR. | last3 = Gupta | first3 = SD. | last4 = Durgapal | first4 = P. | last5 = Iyer | first5 = VK. | last6 = Das | first6 = CJ. | last7 = Shalimar | first7 = SK. | last8 = Acharya | first8 = . | title = Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology. | journal = Cytojournal | volume = 6 | issue =  | pages = 25 | month =  | year = 2010 | doi = 10.4103/1742-6413.58951 | PMID = 20165548 | URL = http://www.biomedsearch.com/nih/Hepatic-epithelioid-hemangioendothelioma-diagnostic-pitfall/20165548.html }}</ref>
*CD31 +ve.
*CD34 +ve.
*Factor VIII +ve.


=See also=
=See also=
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{{Reflist|2}}
{{Reflist|2}}


[[Category:Vascular tumours]]
[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
Michael
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