Difference between revisions of "Angiosarcoma"

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*May arise secondary to chronic lymphedema related to [[invasive breast cancer|breast carcinoma]].
*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>
**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>
*[[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>
*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>

Revision as of 01:45, 17 June 2015

Angiosarcoma
Diagnosis in short

Epithelioid angiosarcoma. H&E stain.
LM atypical cells - usu. spindle cells, occasionally epithelioid; vascular differentiation (abundant capillaries - "red" at low power, +/-cytoplasmic vacuolization, +/-hobnail endothelial cells)
LM DDx Kaposi sarcoma, other vascular tumours
IHC CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve
Molecular +/-MYC amplification
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]
  • Association with radiation - breast,[3] urinary bladder.[4]
  • Liver angiosarcomas are associated with vinyl chloride exposure.[5]
  • Cutaneous angiosarcomas are classically seen on the head and neck of whites over 60 years old.[6]

Gross

  • Red/dark tan lesion.
  • Typically poorly circumscribed.

  • Angiosarcoma. (WC/Yale Rosen)

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

  • Epithelioid angiosarcoma - very low mag. (WC/Nephron)

  • Epithelioid angiosarcoma - intermed mag. (WC/Nephron)

  • Epithelioid angiosarcoma - very high mag. (WC/Nephron)

  • Epithelioid angiosarcoma - CD31 - intermed. mag. (WC/Nephron)

  • Breast - Epithelioid angiosarcoma - high power (SKB)

  • Breast - Epithelioid angiosarcoma - high power (SKB)

  • Breast - Epithelioid angiosarcoma - medium power (SKB)

  • Breast - Angiosarcoma - high grade - high power (SKB)

  • Breast - Angiosarcoma - high grade - high power (SKB)

  • Breast - Angiosarcoma - high grade - high power (SKB)

  • Breast - Angiosarcoma - high power (SKB)

  • Breast - Angiosarcoma - low grade area - encircling fat - high power (SKB)

  • Breast - Angiosarcoma - high power (SKB)

  • Breast - Angiosarcoma - high grade area - high power (SKB)

  • Breast - Angiosarcoma - high power (SKB)

  • Breast - Angiosarcoma - high power (SKB)

  • Breast - Angiosarcoma - low power (SKB)

  • Breast - Angiosarcoma - Medium power (SKB)

  • Breast - Angiosarcoma - low power (SKB)

IHC

  • CD34 +ve.
  • CD31 +ve.
  • ERG +ve [7]
  • FLI-1 +ve.[8]
  • D2-40 +ve/-ve.[9]
  • HHV-8 -ve.

Molecular

  • Amplification of MYC[10] - especially in secondary angiosarcoma.[11]

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. ↑ Chikarmane, SA.; Gombos, EC.; Jagadeesan, J.; Raut, C.; Jagannathan, JP. (Dec 2014). "MRI findings of radiation-associated angiosarcoma of the breast (RAS).". J Magn Reson Imaging. doi:10.1002/jmri.24822. PMID 25504856.
  4. ↑ Bahouth, Z.; Masarwa, I.; Halachmi, S.; Nativ, O. (2015). "Primary angiosarcoma of urinary bladder: 13th reported patient.". Case Rep Oncol Med 2015: 652870. doi:10.1155/2015/652870. PMID 25688314.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ Miettinen, M.; Wang, ZF.; Paetau, A.; Tan, SH.; Dobi, A.; Srivastava, S.; Sesterhenn, I. (Mar 2011). "ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma.". Am J Surg Pathol 35 (3): 432-41. doi:10.1097/PAS.0b013e318206b67b. PMID 21317715.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ Kurisetty, V.; Bryan, BA. (Apr 2013). "Aberrations in Angiogenic Signaling and MYC Amplifications are Distinguishing Features of Angiosarcoma.". Angiol Open Access 1. doi:10.4172/2329-9495.1000102. PMID 25374893.
  11. ↑ Styring, E.; Seinen, J.; Dominguez-Valentin, M.; Domanski, HA.; Jönsson, M.; von Steyern, FV.; Hoekstra, HJ.; Suurmeijer, AJ. et al. (Jul 2014). "Key roles for MYC, KIT and RET signaling in secondary angiosarcomas.". Br J Cancer 111 (2): 407-12. doi:10.1038/bjc.2014.359. PMID 24983371.
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