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.
  • Mitoses.
  • Cytoplasmic vacuoles.
    • Cells trying to form lumina - embryologic.

Notes:

DDx:

Images

IHC

  • CD34 +ve.
  • CD31 +ve.
  • FLI-1 +ve.[5]
  • D2-40 +ve/-ve.[6]
  • HHV-8 -ve.

See also

References

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.