Abstract
BackgroundThe “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.CaseWe 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.ConclusionEven 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 slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1508554852942125
Highlights
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
We report a case of vulvar “proximal-type” ephitelioid sarcoma accompanied by regional lymph node metastatic repetitions in a 63 old woman
Epithelioid sarcoma was first described by Enzinger in 1970 [3]. It is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass and originating in distal and proximal extremities of adolescents and young adults. It shows a rapid clinical evolution and tents to spread to the body in particular to the regional lymph nodes, to the lung and in the abdomen
Summary
Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of 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
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