View source for Angiosarcoma

{{ Infobox diagnosis
| Name       = {{PAGENAME}}
| Image      = Epithelioid_angiosarcoma_-_very_high_mag.jpg 
| Width      =
| Caption    = Epithelioid angiosarcoma. [[H&E stain]].
| Micro      = atypical cells - usu. spindle cells, occasionally epithelioid; vascular differentiation (abundant capillaries - "red" at low power, +/-cytoplasmic vacuolization, +/-[[hobnail]] endothelial cells)
| Subtypes   = 
| LMDDx      = [[Kaposi sarcoma]], [[anastomosing hemangioma]], other [[vascular tumours]]
| Stains     = 
| IHC        = CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve
| EM         =
| Molecular  = +/-MYC amplification
| IF         =
| Gross      = red lesion{{fact}}
| Grossing   =
| Site       = [[skin]], [[head and neck pathology|head and neck]], elsewhere
| Assdx      =
| Syndromes  = Stewart–Treves syndrome
| Clinicalhx = +/-chronic lymphedema, vinyl chloride exposure (liver angiosarcoma)
| Signs      =
| Symptoms   =
| Prevalence = uncommon
| Bloodwork  =
| Rads       =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    =
}}
'''Angiosarcoma''' is an uncommon malignant [[vascular tumour]].

==General==
*Malignant tumour - general has a poor 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>

Epidemiology:
*May arise secondary to chronic lymphedema related to [[invasive breast cancer|breast carcinoma]].
**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 | url=http://www.nejm.org/doi/full/10.1056/NEJMicm071344}}</ref>
*Association with radiation - breast,<ref name=pmid25504856>{{Cite journal  | last1 = Chikarmane | first1 = SA. | last2 = Gombos | first2 = EC. | last3 = Jagadeesan | first3 = J. | last4 = Raut | first4 = C. | last5 = Jagannathan | first5 = JP. | title = MRI findings of radiation-associated angiosarcoma of the breast (RAS). | journal = J Magn Reson Imaging | volume =  | issue =  | pages =  | month = Dec | year = 2014 | doi = 10.1002/jmri.24822 | PMID = 25504856 }}</ref> [[urinary bladder]].<ref name=pmid25688314>{{Cite journal  | last1 = Bahouth | first1 = Z. | last2 = Masarwa | first2 = I. | last3 = Halachmi | first3 = S. | last4 = Nativ | first4 = O. | title = Primary angiosarcoma of urinary bladder: 13th reported patient. | journal = Case Rep Oncol Med | volume = 2015 | issue =  | pages = 652870 | month =  | year = 2015 | doi = 10.1155/2015/652870 | PMID = 25688314 }}</ref>
*[[Hepatic angiosarcoma|Liver angiosarcoma]]s are associated with vinyl chloride exposure.<ref>{{Ref PCPBoD8|212}}</ref>
*Cutaneous angiosarcomas are classically seen on the head and neck of whites over 60 years old.<ref name=pmid>{{Cite journal  | last1 = Albores-Saavedra | first1 = J. | last2 = Schwartz | first2 = AM. | last3 = Henson | first3 = DE. | last4 = Kostun | first4 = L. | last5 = Hart | first5 = A. | last6 = Angeles-Albores | first6 = D. | last7 = Chablé-Montero | first7 = F. | title = Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007. | journal = Ann Diagn Pathol | volume = 15 | issue = 2 | pages = 93-7 | month = Apr | year = 2011 | doi = 10.1016/j.anndiagpath.2010.07.012 | PMID = 21190880 }}</ref>

==Gross==
*Red/dark tan lesion.
*Typically poorly circumscribed.

<gallery>
Image:Angiosarcoma (5617087462).jpg | Angiosarcoma. (WC/Yale Rosen)
</gallery>
==Microscopic==
Features:
*Spindle cell lesion.
**Occasionally an epithelioid lesion.
*Very many small capillaries of irregular shape lined with:
**Pleomorphic nuclei - '''important'''.
***May have [[hobnail morphology]].
**Usually "red" at low power - due to many RBCs - '''important'''.
*Mitoses.
*Cytoplasmic vacuoles.
**Cells trying to form lumina - embryologic.

Notes:
*Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble ''[[melanoma]]'' or carcinoma (e.g. ''[[hepatocellular carcinoma]]'', ''[[urothelial carcinoma]]''<ref name=pmid25929352>{{cite journal |authors=Matoso A, Epstein JI |title=Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases |journal=Am J Surg Pathol |volume=39 |issue=10 |pages=1377–82 |date=October 2015 |pmid=25929352 |doi=10.1097/PAS.0000000000000444 |url=}}</ref>).

DDx:
*Atypical vascular lesion.
*[[Kaposi sarcoma]].
*[[Anastomosing hemangioma]] - especiallly in the [[kidney]].
*Poorly differentiated carcinoma, e.g. [[urothelial carcinoma]].
*Other [[vascular tumours]].

===Images===
====Case 1====
<gallery>
Image:Epithelioid_angiosarcoma_-_very_low_mag.jpg | Epithelioid angiosarcoma - very low mag. (WC/Nephron)
Image:Epithelioid_angiosarcoma_-_intermed_mag.jpg | Epithelioid angiosarcoma - intermed mag. (WC/Nephron)
Image:Epithelioid_angiosarcoma_-_very_high_mag.jpg | Epithelioid angiosarcoma - very high mag. (WC/Nephron)
Image:Epithelioid_angiosarcoma_-_CD31_-_intermed_mag.jpg | Epithelioid angiosarcoma - CD31 - intermed. mag. (WC/Nephron)
</gallery>
====Case 2====
<gallery>
Image: Renal angiosarcoma -- intermed mag.jpg | Angiosarcoma (kidney) - low mag.
Image: Renal angiosarcoma - alt -- intermed mag.jpg | Angiosarcoma (kidney) - low mag.
Image: Renal angiosarcoma -- high mag.jpg | Angiosarcoma (kidney) - high mag.
Image: Renal angiosarcoma -- very high mag.jpg | Angiosarcoma (kidney) - very high mag.

Image: Renal angiosarcoma in fat -- intermed mag.jpg | Angiosarcoma (kidney) - low mag.
Image: Renal angiosarcoma in fat - alt -- intermed mag.jpg | Angiosarcoma (kidney) - low mag.
Image: Renal angiosarcoma in fat -- high mag.jpg | Angiosarcoma (kidney) - high mag.
</gallery>

====Additional====
<gallery>
Image:Breast Angiosarcoma Epithelioid HP2 CTR.jpg|Breast - Epithelioid angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma Epithelioid HP CTR.jpg|Breast - Epithelioid angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma Epithelioid MP CTR.jpg|Breast - Epithelioid angiosarcoma - medium power (SKB)
Image:Breast Angiosarcoma HighGrade Breast HP PA.JPG|Breast - Angiosarcoma - high grade - high power (SKB)
Image:Breast Angiosarcoma HighGrade Breast PA.JPG|Breast - Angiosarcoma - high grade - high power (SKB)
Image:Breast Angiosarcoma HighGrade HP PA.JPG|Breast - Angiosarcoma - high grade - high power (SKB)
Image:Breast Angiosarcoma HP3 SNP.jpg|Breast - Angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma LowGradeArea FatInvasion MP CTR.jpg|Breast - Angiosarcoma - low grade area - encircling fat - high power (SKB)
Image:Breast Angiosarcoma HP SNP.jpg|Breast - Angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma HighGradeArea MP CTR.jpg|Breast - Angiosarcoma - high grade area - high power (SKB)
Image:Breast Angiosarcoma HP PA.JPG|Breast - Angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma HighGrade PA.JPG|Breast - Angiosarcoma - high power (SKB)
Image:Breast Angiosarcoma LP SNP.jpg|Breast - Angiosarcoma - low power (SKB)
Image:Breast Angiosarcoma MP SNP.jpg|Breast - Angiosarcoma - Medium power (SKB)
Image:Breast Angiosarcoma LP CTR.jpg|Breast - Angiosarcoma - low power (SKB)
</gallery>

==IHC==
*CD34 +ve.
*CD31 +ve.
*ERG +ve <ref>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Wang | first2 = ZF. | last3 = Paetau | first3 = A. | last4 = Tan | first4 = SH. | last5 = Dobi | first5 = A. | last6 = Srivastava | first6 = S. | last7 = Sesterhenn | first7 = I. | title = ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. | journal = Am J Surg Pathol | volume = 35 | issue = 3 | pages = 432-41 | month = Mar | year = 2011 | doi = 10.1097/PAS.0b013e318206b67b | PMID = 21317715 }}
</ref>
*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.

==Molecular==
*Amplification of MYC<ref name=pmid25374893>{{Cite journal  | last1 = Kurisetty | first1 = V. | last2 = Bryan | first2 = BA. | title = Aberrations in Angiogenic Signaling and MYC Amplifications are Distinguishing Features of Angiosarcoma. | journal = Angiol Open Access | volume = 1 | issue =  | pages =  | month = Apr | year = 2013 | doi = 10.4172/2329-9495.1000102 | PMID = 25374893 }}</ref> - especially in secondary angiosarcoma.<ref name=pmid24983371>{{Cite journal  | last1 = Styring | first1 = E. | last2 = Seinen | first2 = J. | last3 = Dominguez-Valentin | first3 = M. | last4 = Domanski | first4 = HA. | last5 = Jönsson | first5 = M. | last6 = von Steyern | first6 = FV. | last7 = Hoekstra | first7 = HJ. | last8 = Suurmeijer | first8 = AJ. | last9 = Nilbert | first9 = M. | title = Key roles for MYC, KIT and RET signaling in secondary angiosarcomas. | journal = Br J Cancer | volume = 111 | issue = 2 | pages = 407-12 | month = Jul | year = 2014 | doi = 10.1038/bjc.2014.359 | PMID = 24983371 }}</ref>

==See also==
*[[Kaposi sarcoma]].
*[[Vascular tumours]].

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

[[Category:Diagnosis]]
[[Category:Vascular tumours]]