Difference between revisions of "Angiosarcoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Epithelioid_angiosarcoma_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Epithelioid angiosarcoma. [[H&E stain]]. | |||
| Micro = atypical spindle cell lesions (occasional epithelioid), vascular differentiation (abundant capillaries, cytoplasmic vacuolization, hobnail endothelial cells) | |||
| Subtypes = | |||
| LMDDx = [[Kaposi sarcoma]], other [[vascular tumours]] | |||
| Stains = | |||
| IHC = CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve | |||
| EM = | |||
| Molecular = | |||
| 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]]. | '''Angiosarcoma''' is an uncommon malignant [[vascular tumour]]. | ||
Revision as of 20:22, 9 October 2013
Angiosarcoma | |
---|---|
Diagnosis in short | |
Epithelioid angiosarcoma. H&E stain. | |
| |
LM | atypical spindle cell lesions (occasional epithelioid), vascular differentiation (abundant capillaries, cytoplasmic vacuolization, hobnail endothelial cells) |
LM DDx | Kaposi sarcoma, other vascular tumours |
IHC | CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve |
Gross | red lesion[citation needed] |
Site | skin, head and neck, elsewhere |
| |
Syndromes | Stewart–Treves syndrome |
| |
Clinical history | +/-chronic lymphedema, vinyl chloride exposure (liver angiosarcoma) |
Prevalence | uncommon |
Prognosis | poor |
Angiosarcoma is an uncommon malignant vascular tumour.
General
- Malignant tumour - general has a poor prognosis.[1]
Epidemiology:
- May arise secondary to chronic lymphedema related to breast carcinoma.
- Known as Stewart–Treves syndrome.[2]
- Liver angiosarcomas are associated with vinyl chloride exposure.[3]
- Cutaneous angiosarcomas are classically seen on the head and neck of whites over 60 years old.[4]
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.
- Pleomorphic nuclei - important.
- Mitoses.
- Cytoplasmic vacuoles.
- Cells trying to form lumina - embryologic.
Notes:
- Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble melanoma or hepatocellular carcinoma.
DDx:
- Atypical vascular lesion.
- Kaposi sarcoma.
- Poorly differentiated carcinoma.
Images
IHC
See also
References
- ↑ Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (May 2010). "Angiosarcoma". Lancet Oncol. doi:10.1016/S1470-2045(10)70023-1. PMID 20537949.
- ↑ Pincus, LB.; Fox, LP. (Aug 2008). "Images in clinical medicine. The Stewart-Treves syndrome.". N Engl J Med 359 (9): 950. doi:10.1056/NEJMicm071344. PMID 18753651. http://www.nejm.org/doi/full/10.1056/NEJMicm071344.
- ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 212. ISBN 978-1416054542.
- ↑ Albores-Saavedra, J.; Schwartz, AM.; Henson, DE.; Kostun, L.; Hart, A.; Angeles-Albores, D.; Chablé-Montero, F. (Apr 2011). "Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007.". Ann Diagn Pathol 15 (2): 93-7. doi:10.1016/j.anndiagpath.2010.07.012. PMID 21190880.
- ↑ Rossi, S.; Orvieto, E.; Furlanetto, A.; Laurino, L.; Ninfo, V.; Dei Tos, AP. (May 2004). "Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody.". Mod Pathol 17 (5): 547-52. doi:10.1038/modpathol.3800065. PMID 15001993.
- ↑ Kahn, HJ.; Bailey, D.; Marks, A. (Apr 2002). "Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas.". Mod Pathol 15 (4): 434-40. doi:10.1038/modpathol.3880543. PMID 11950918.