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Vascular tumours (view source)

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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>
 
====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.
====Soft tissue====
Several types are seen in soft tissue:<ref>{{Ref WMSP|602}}</ref>
*Capillary.
*Cavernous.
*Arteriovenous.
*Venous.
*Intramuscular.
*Synovial.


===Microscopic===
==Lymphangioma==
Features:
*[[AKA]] ''cystic hygroma''.<ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*Abundance of benign small blood vessels. (???)


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)].
==Kaposi sarcoma==
===General===
===General===
*Not really a sarcoma.
*Benign.
*Caused by [[Human herpesvirus-8]] (HHV-8).
*Classically in the left neck.<ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*Associated with immunodeficiency, e.g. [[HIV]]/AIDS.
*May be seen in [[Turner syndrome]].


===Stages===
Treatment:
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>
*Surgical excision.
#Patch stage.
#Plaque stage.
#Nodular stage.
#Exophytic stage.
#Infiltrative stage.
#Lymphadenopathic stage.


===Microscopic===
===Microscopic===
Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>- '''key feature'''.
Features:<ref name=Ref_WMSP489>{{Ref WMSP|489}}</ref><ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*+/-Nuclear atypia.
*Thin-walled channels lined by endothelium.
*[[Hyaline globules]] (intracytoplasmic)<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'''.
*+/-Eosinophilic intraluminal material.
*+/-Hemosiderin deposits.
*+/-Clusters of intraluminal lymphocytes.
*+/-Occasional [[RBC]]s.


DDx:
DDx:
*Angiosarcoma (have many mitoses, nuclear atypia).
*[[Hemangioma]].
*Masson's hemangioma (Intravascular papillary endothelial hyperplasia).
 
Notes:
*Hyaline globules have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/>
*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>
**Image: [http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].


Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
*[http://www.webpathology.com/image.asp?n=5&Case=412 Lymphangioma - low mag. (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
*[http://www.webpathology.com/image.asp?case=412&n=6 Lymphangioma - high mag. (webpathology.com)].


===IHC===
===IHC===
*CD31 +ve.  
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>
*CD34 +ve.  
*D2-40 +ve.
*[[HHV-8]] +ve.
 
==Kaposi sarcoma==
{{Main|Kaposi sarcoma}}


==Masson hemangioma==
==Masson hemangioma==
===General===
*Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
*[[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>  
*Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.<ref name=pmid15865098/>
*[[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>
===Microscopic===
{{Main|intravascular papillary endothelial hyperplasia}}
Features:
*Well-circumscribed - '''key (low power) feature'''.  
*Abundant small vascular channels with benign endothelium.
 
Notes:
*Looks like [[Kaposi sarcoma]] at high power.


==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.
*D2-40 +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>


==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===
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>
*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.


Image:
==Retiform hemangioendothelioma==
*[http://en.wikipedia.org/wiki/File:Epithelioid_hemangioendothelioma_EHE.Image5.jpg Epithelioid hemangioendothelioma (WP)].
{{Main|Retiform hemangioendothelioma}}


===IHC===
==Intimal sarcoma==
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|Intimal sarcoma}}
*CD31 +ve.
*CD34 +ve.
*Factor VIII +ve.


=See also=
=See also=
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*[[Vasculitides]].
*[[Vasculitides]].
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Cisterna chyli]].


=References=
=References=
{{Reflist|2}}
{{Reflist|2}}


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