Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist

Gian Franco Zannoni, Angiolo Gadducci

Risultato della ricerca: Contributo in rivistaArticolo in rivista

1 Citazioni (Scopus)

Abstract

Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a molecular alteration leading to expression of melanocytic markers, or from pericytic cells. The majority of cases are benign, but a small subset behaves in a malignant fashion. Unfortunately, given their rarity, reliable criteria for predicting malignancy have yet to be established. PEComas of the female genital tract account for nearly 25% of PEComas of all body sites, and the most common site of occurrence is the uterine corpus; less common sites include the cervix, adnexa, vagina/vulva or broad or round ligament exceptionally. The present review aims to elucidate the clinical, pathological and molecular features of gynecological PEComas. Moreover, prognostic and therapeutic implications are also discussed.
Lingua originaleEnglish
pagine (da-a)1-12
Numero di pagine12
RivistaGYNECOLOGIC ONCOLOGY REPORT
Volume33
DOI
Stato di pubblicazionePubblicato - 2020

Keywords

  • Cervical PEComas
  • Melanocytic markers
  • Uterine perivascular epithelioid cell tumors (PEComas)
  • Ovarian PEComas
  • Myogenic markers

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