Vulvar “proximal-type” epithelioid sarcoma: report of a case and review of the literature

Diagnostic Pathology

Enrico Vizza⁴  Emilio Piccione³  Chiara Scelzo³  Letizia Perracchio²  Maria Saltari³  Giacomo Corrado¹  Lodovico Patrizi³ 

[1] Department of Oncological Surgery, Gynecologic Oncology Unit, “Regina Elena” National Cancer Institute, Via Elio Chianesi 53, Rome, 00144, Italy;Pathology Department, “Regina Elena” National Cancer Institute, Rome, Italy;Surgery Department, Gynecology Section and Obstetrics, Tor Vergata University, Rome, Italy;Surgery Department, Gynecologic Oncology Unit, “Regina Elena” National Cancer Institute, Rome, Italy

关键词: Vulvar cancer;    Radical vulvectomy;    Vulvar “proximal-type” epithelioid sarcoma;   

Others  :  805999
DOI  :  10.1186/1746-1596-8-122

 received in 2013-03-12, accepted in 2013-06-21,  发布年份 2013

【 摘 要 】

Background

The “proximal-type” epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior.

Case

We report of a case of a 63 years old woman with a vulvar “proximal-type” epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease.

Conclusion

Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy.

Virtual slides

The virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/1508554852942125 webcite

【 授权许可】

   
2013 Patrizi et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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