Skip to main content

Advertisement

Log in

Angiosarcoma Developing in a Vagal Schwannoma: A Rare Case Report

  • Case Report
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Angiosarcoma arising in a schwannoma is extremely rare with only fourteen cases having been reported in the literature to the best of our knowledge. Amongst these fourteen, only five cases developed from vagal schwannoma. We describe a case of epithelioid angiosarcoma arising in a long standing vagal schwannoma in a 41 years male patient. Grossly the tumor was well encapsulated with variegated cut surface. On microscopy the tumor had two distinct components composed of benign schwannoma and malignant angiosarcoma which were further confirmed by immunohistochemistry. On further work up, he was found to have multiple distant metastases. This is the sixth reported case of angiosarcoma arising in a vagal schwannoma. The proposed histogenesis of this rare transformation, its prognostic factors and a review of literature regarding this entity is discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

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

References

  1. McMenamin ME, Fletcher CD. Expanding the spectrum of malignant change in Schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. Am J Surg Pathol. 2001;25(1):13–25.

    Article  CAS  PubMed  Google Scholar 

  2. Mentzel T, Katenkamp D. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases. Histopathology. 1999;35(2):114–20.

    Article  CAS  PubMed  Google Scholar 

  3. Scheithauer B, Louis D, Hunter S, Woodruff JM, et al. Schwannoma. In: Louis D, Ohgaki H, Wiestler O, Cavenee W, editors. WHO classification of tumours of the central nervous system. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2007. p. 152–5.

    Google Scholar 

  4. Woodruff JM, Selig AM, Crowley K, Allen PW. Schwannoma (neurilemoma) with malignant transformation, A rare, distinctive peripheral nerve tumor. Am J Surg Pathol. 1994;18(9):882–95.

    Article  CAS  PubMed  Google Scholar 

  5. Trassard M, Le Doussal V, Bui BN, Coindre JM. Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve. Am J Surg Pathol. 1996;20(11):1412–7.

    Article  CAS  PubMed  Google Scholar 

  6. Rückert RI, Fleige B, Rogalla P, Woddruff JM. Schwannoma with angiosarcoma, report of a case and comparison with other types of nerve tumors with angiosarcoma. Cancer. 2000;89:1577–85.

    Article  PubMed  Google Scholar 

  7. Shundo Y, Ota S, Inaba H, et al. Angiosarcoma arising from a solitary schwannoma of the chest wall [in Japanese]. Kyobu Geka. 2002;55:847–51.

    CAS  PubMed  Google Scholar 

  8. Ito T, Tsutsumi T, Ohno K, et al. Intracranial angiosarcoma arising from a schwannoma. J Laryngol Otol. 2007;121:68–71.

    Article  CAS  PubMed  Google Scholar 

  9. Lee FY, Wen MC, Wang J. Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review. Hum Pathol. 2007;38:1096–101.

    Article  CAS  PubMed  Google Scholar 

  10. Li C, Chen Y, Zhang H, Zheng X, Wang J. Epithelioid angiosarcoma arising in schwannoma: report of three Chinese cases with review of the literature. Pathol Int. 2012;62(7):500–5.

    Article  PubMed  Google Scholar 

  11. Weiss S, Goldblum J, editors. Enzinger and Weiss’s Soft tissue tumors. 4th ed. USA: Mosby; 2001. Chapter 30-Benign tumors of peripheral nerves. pp. 1111–1208.

  12. DeYoung BR, Wick MR, Fitzgibbon JF, et al. CD31: an immunospecific marker for endothelial differentiation in human neoplasms. Appl Immunohistochem. 1993;1:97.

    CAS  Google Scholar 

  13. Orchard GE, Zelger B, Jones EW, et al. An immunocytochemical assessment of 19 cases of cutaneous angiosarcoma. Histopathology. 1996;28:235.

    Article  CAS  PubMed  Google Scholar 

Download references

Conflict of interest

The authors declare that there are no conflicts of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Seema Rao.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mahajan, V., Rao, S., Gupta, P. et al. Angiosarcoma Developing in a Vagal Schwannoma: A Rare Case Report. Head and Neck Pathol 9, 405–411 (2015). https://doi.org/10.1007/s12105-014-0577-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-014-0577-x

Keywords

Navigation