Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review

Angela Musella, Francesca De Felice, A. Kyriacos Kyriacou, Francesco Barletta, Filippo Maria Di Matteo, Claudia Marchetti, Luciano Izzo, Marco Monti, Pierluigi Benedetti Panici, Adriano Redler, Vito D'Andrea

Risultato della ricerca: Contributo in rivistaArticolo in rivista


Background: Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumor. Gynecological PEComas account for just over one-fourth of the overall PEComa cases reported in the literature. Surgery is the most recommended primary treatment while adjuvant therapy is generally reserved for high-risk cases. However, the best management of this neoplasia has not been well established, primarily because of the paucity of cases described to date. Objectives: The aim of this systematic review is to summarize what is known thus far regarding the etiopathogenesis, clinical and pathologic features of PEComas, focusing also on the most valid treatment options for uterine cases. Data sources: Pubmed articles on PEComas published in various journals over the past 70 years were analyzed. Conclusions and key findings: Although the optimal treatment of gynecological PEComas is controversial, surgical resection remains the cornerstone. The use of adjuvant treatment is warranted in high risk patients to increase disease control. A multidisciplinary approach should be key in treatment decision-making regarding gynecological PEComas.
Lingua originaleEnglish
pagine (da-a)1-5
Numero di pagine5
RivistaInternational Journal of Surgery
Stato di pubblicazionePubblicato - 2015


  • Gynecological
  • Neoplasm
  • PEComa
  • Perivascular epithelioid cell
  • Uterus


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