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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.
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| ***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>
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| Hemangiomas to remember - if you're only going remember a few:
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| *''Glomeruloid'', ''infantile'', ''caverous'', ''capillary'', ''arteriovenous'', ''venous'' and ''intramuscular''.
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| ====Childhood====
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| 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>
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| *Tufted - small clusters of blood vessels.
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| *Microvenular hemangioma.
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| *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>
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| *Epithelioid hemangioma.
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| *Targetoid hemosideric hemangioma.
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| *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>
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| ====Soft tissue====
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| Several types are seen in soft tissue:<ref>{{Ref WMSP|602}}</ref>
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| *Capillary.
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| *Cavernous.
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| *Arteriovenous.
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| *Venous.
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| *Intramuscular.
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| *Synovial.
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| ===Microscopic===
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| Features:
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| *Channels lined by endothelium containing [[RBC]]s.
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| DDx:
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| *[[Lymphangioma]].
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| Images:
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| *[[WC]]:
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| **[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_intermed_mag.jpg Capillary hemangioma - intermed. mag. (WC)].
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| **[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_very_high_mag.jpg Capillary hemangioma - very high mag. (WC)].
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| *www:
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| **[http://path.upmc.edu/cases/case277.html Diffuse hemangioma - several images (upmc.edu)].
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| ===IHC===
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| *CD31 +ve.
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| *D2-40 -ve.<ref name=pmid11950918/>
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| ''Juvenile hemangioma'':<ref name=pmid10665907/>
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| *GLUT-1 +ve.
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| ==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. |
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| ==Kaposi sarcoma== | | ==Kaposi sarcoma== |
| *Abbreviated ''KS''.
| | {{Main|Kaposi sarcoma}} |
| ===General===
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| *Caused by [[Human herpesvirus-8]] (HHV-8).
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| *In the North American context, it is often associated with immunodeficiency, e.g. [[HIV]]/AIDS.
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| Interesting note:
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| *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>
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| ====Stages====
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| 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>
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| #Patch stage.
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| #Plaque stage.
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| #Nodular stage.
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| #Exophytic stage.
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| #Infiltrative stage.
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| #Lymphadenopathic stage.
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| Note:
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| *The first three are the classic ones.
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| ====Type or form====
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| 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>
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| *Classic = old men Mediterranean or Ashkenazi Jew.
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| *Endemic = African infants and young males.
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| *Immunosuppression-associated or transplant-associated - iatrogenic.
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| *AIDS-associated.
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| ===Microscopic===
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| Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>
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| *Vascular lesion (abundant [[RBC]]s) with:
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| **+/-"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>
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| ***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>
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| **+/-Spindle cells with minimal nuclear atypia.
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| **[[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>
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| *+/-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>
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| **Pale pink globs (that are paler than RBCs) - '''important feature'''.
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| *+/-Hemosiderin deposits.
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| *+/-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>
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| Notes:
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| *[[Hyaline globules]] have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/>
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| DDx:
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| *[[Angiosarcoma]] - have many mitoses, nuclear atypia, RBC extravasation not common.
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| *[[Masson's hemangioma]] - [[AKA]] ''intravascular papillary endothelial hyperplasia''.
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| *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>
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| **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>
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
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| *[http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].
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| ===Stains===
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| *PAS +ve -- hyaline globules.
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| ===IHC===
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| *CD31 +ve.
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| *CD34 +ve.
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| *[[HHV-8]] +ve.
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| ==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.
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| *Histomorphologically may be confused with low-grade [[angiosarcoma]] or other soft tissue sarcomas.<ref name=pmid15865098/>
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| ===Microscopic===
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| Features:
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| *Well-circumscribed - '''key (low power) feature'''.
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| *Abundant small vascular channels with benign endothelium.
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| *+/-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>
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| Notes:
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| *Looks like [[Kaposi sarcoma]] at high power.
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| Images:
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| *[http://path.upmc.edu/cases/case544.html Masson hemangioma - several images (upmc.edu)].
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| ==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>
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| *Classically on the scalp or head & neck.
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| Epidemiology:
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| *May arise secondary to chronic lymphedema related to breast carcinoma.
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| **Known as ''Stewart–Treves syndrome''.<ref name=pmid18753651>{{Cite journal | last1 = Pincus | first1 = LB. | last2 = Fox | first2 = LP. | title = Images in clinical medicine. The Stewart-Treves syndrome. | journal = N Engl J Med | volume = 359 | issue = 9 | pages = 950 | month = Aug | year = 2008 | doi = 10.1056/NEJMicm071344 | PMID = 18753651 }}</ref>
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| *[[Liver]] angiosarcomas are associated with vinyl chloride exposure.<ref>{{Ref PCPBoD8|212}}</ref>
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| ===Microscopic===
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| Features:
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| *Very many small capillaries of irregular shape lined with:
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| **Pleomorphic nuclei.
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| ***May have hobnail morphology.
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| *Mitoses.
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| *Cytoplasmic vacuoles.
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| **Cells trying to form lumina - embryologic.
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| Notes:
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| *Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble ''[[melanoma]]'' or ''[[hepatocellular carcinoma]]''.
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_low_mag.jpg Epithelioid angiosarcoma - very low mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_intermed_mag.jpg Epithelioid angiosarcoma - intermed mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_high_mag.jpg Epithelioid angiosarcoma - very high mag. (WC)].
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| ===IHC===
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| *CD34 +ve.
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| *CD31 +ve.
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| *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>
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| *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>
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| *HHV-8 -ve.
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| ==Kaposiform hemangioendothelioma== | | ==Kaposiform hemangioendothelioma== |
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| ==Epithelioid hemangioendothelioma== | | ==Epithelioid hemangioendothelioma== |
| *Should '''not''' be confused with ''[[epithelioid hemangioma]]''.
| | {{Main|Epithelioid hemangioendothelioma}} |
| ===General===
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| *Malignant.<ref name=Ref_WMSP603>{{Ref WMSP|603}}</ref>
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| *Adults - wide age range.
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| *Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/>
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| ===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:
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| **Abundant pale eosinophilic cytoplasm.
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| **Cytoplasmic vacuolation (some cells) - [[AKA]] "blister cells" - '''key feature'''.
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| ***May form lumen and have RBC within.
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| **Vesicular nucleus with prominent nucleolus in some cells.
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| *Tuft-like projections into capillaries.
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| *Tumour cells may be in well-circumscribed paucicellular nodules ''or'' more cellular poorly formed aggregates.
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|
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| DDx:
| | ==Intimal sarcoma== |
| *[[Angiosarcoma]], epithelioid.
| | {{Main|Intimal sarcoma}} |
| *[[Hemangioma]].
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| Images:
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| *[http://en.wikipedia.org/wiki/File:Epithelioid_hemangioendothelioma_EHE.Image5.jpg Epithelioid hemangioendothelioma (WP)].
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| *[http://www.flickr.com/photos/pulmonary_pathology/5600772691/in/photostream/ Epithelioid hemangioendothelioma - low mag. (flickr.com/Rosen)].
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| *[http://www.flickr.com/photos/pulmonary_pathology/5601354962/ Epithelioid hemangioendothelioma - high mag. (flickr.com/Rosen)].
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| *[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802170854495 Epithelioid hemangioendothelioma (surgicalpathologyatlas.com)].
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| ===IHC===
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| 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>
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| *CD31 +ve.
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| *CD34 +ve.
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| *Factor VIII +ve.
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| =See also= | | =See also= |
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| {{Reflist|2}} | | {{Reflist|2}} |
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| | [[Category:Vascular tumours]] |
| [[Category:Soft tissue lesions]] | | [[Category:Soft tissue lesions]] |
