Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

A case of high-risk penile epithelioid hemangioendothelioma

Nature Reviews Urology volume 6, pages 223–227 (2009)Cite this article

Abstract

Background. A 48-year-old, previously healthy man presented to his local urologist with several small, violaceous, indurated lesions along the shaft of his penis and glans, which caused discomfort and penile curvature. Four weeks previously, the lesions were three small painless blemishes. Peyronie disease was initially diagnosed, but the disease progressed despite steroid treatment. Following the results of a biopsy, multifocal angiosarcoma was diagnosed. CT of the chest, abdomen and pelvis was negative for metastatic disease. At this point the patient was referred to a specialist oncology center for further management.

Investigations. At the specialty center, the patient underwent physical examination, additional excisional biopsies, and Pet–ct.

Diagnosis. Penile epithelioid hemangioendothelioma, which is a multifocal, atypical epithelioid vascular tumor.

Management. Single-agent chemotherapy using liposomal doxorubicin hydrochloride for eight cycles, followed by 63 Gy of adjuvant external beam radiation therapy to the penis fractionated over 35 treatments.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Preoperative photographs of the patient's penis.
Figure 2: Photomicrographs of the excisional biopsy samples, stained with hematoxylin and eosin.
Figure 3: Photographs of the penis 18 months after initiation of treatment.

References

  1. Fetsch, J. F., Sesterhenn, I. A., Miettinen, M. & Davis, C. J. Jr. Epithelioid hemangioma of the penis: a clinicopathologic and immunohistochemical analysis of 19 cases, with special reference to exuberant examples often confused with epithelioid hemangioendothelioma and epithelioid angiosarcoma. Am. J. Surg. Pathol. 28, 523–533 (2004).

    Article  PubMed  Google Scholar 

  2. Hauck, E. W. et al. Epithelioid sarcoma of the penis—a rare differential diagnosis of Peyronie's disease. Int. J. Impot. Res. 15, 378–382 (2003).

    Article  CAS  PubMed  Google Scholar 

  3. Usta, M. F., Adams, D. M., Zhang, J. W., Davis, R. & Hellstrom, W. J. Penile epithelioid sarcoma and the case for a histopathological diagnosis in Peyronie's disease. BJU Int. 91, 519–521 (2003).

    Article  CAS  PubMed  Google Scholar 

  4. Enzinger, F. M. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 26, 1029–1041 (1970).

    Article  CAS  PubMed  Google Scholar 

  5. Pueblitz, S., Mora-Tiscareño, A., Meneses-García, A. A., Lozano-Lozano, G. & Ocampo-delCarpio, O. Epithelioid sarcoma of penis. Urology 28, 246–249 (1986).

    Article  CAS  PubMed  Google Scholar 

  6. Deyrup, A. T., Tighiouart, M., Montag, A. G. & Weiss, S. W. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am. J. Surg. Pathol. 32, 924–927 (2008).

    Article  PubMed  Google Scholar 

  7. Fury, M. G., Antonescu, C. R., Van Zee, K. J., Brennan, M. F. & Maki, R. G. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J. 11, 241–247 (2005).

    Article  PubMed  Google Scholar 

  8. Mentzel, T., Beham, A., Calonje, E., Katenkamp, D. & Fletcher, C. D. Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. Am. J. Surg. Pathol. 21, 363–374 (1997).

    Article  CAS  PubMed  Google Scholar 

  9. Weiss, S. W. & Enzinger, F. M. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50, 970–981 (1982).

    Article  CAS  PubMed  Google Scholar 

  10. Fletcher, C. D. M., Unni, K. K. & Mertens, F. WHO Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press: Lyon, France (2002).

    Google Scholar 

  11. Gharajeh, A., Siemens, D. R., Isotalo, P. A., Nascimento, A. G. & Borth, C. S. Multifocal penile epithelioid hemangioendothelioma masquerading as superficial penile vein thrombosis. Urology 68, 673e1–e3 (2006).

    Article  Google Scholar 

  12. Wen, C. C., Munarriz, R. & Goldstein, I. Three-chamber priapism in a patient with primary epithelioid hemangioendothelioma of penis. Urology 64, 156–158 (2004).

    Article  PubMed  Google Scholar 

  13. Zastrow, S., Baretton, G. B. & Wirth, M. P. Multifocal recurring epithelioid hemangioendothelioma of the penis. Urology 71, 351e9–10 (2008).

    Article  Google Scholar 

  14. Kamat, A. M., Plager, C., Tamboli, P., Tran, N. & Pettaway, C. A. Metastatic epithelioid hemangioendothelioma of the penis managed with surgery and interferon-alpha. J. Urol. 171, 1886–1887 (2004).

    Article  PubMed  Google Scholar 

  15. Deutsch, M., Leen, R. L. & Mercado, R. Jr . Hemangioendothelioma of the penis with late appearing metastases: report of a case with review of the literature. J. Surg. Oncol. 5, 27–34 (1973).

    Article  CAS  PubMed  Google Scholar 

  16. Celikel, C. et al. Epithelioid hemangioendothelioma with multiple organ involvement. APMIS 115, 881–888 (2007).

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Memorial Sloan–Kettering Cancer Center, New York, NY, USA

    Alexei Wedmid, Timothy A. Masterson, Robert G. Maki & Paul Russo

Authors
  1. Alexei Wedmid
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Timothy A. Masterson
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Robert G. Maki
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Paul Russo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Paul Russo.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wedmid, A., Masterson, T., Maki, R. et al. A case of high-risk penile epithelioid hemangioendothelioma. Nat Rev Urol 6, 223–227 (2009). https://doi.org/10.1038/nrurol.2009.26

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1038/nrurol.2009.26

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing