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Perivascular epithelioid tumours (PEComas) of the gynaecological tract
  1. Niamh Conlon1,
  2. Robert A Soslow1,
  3. Rajmohan Murali1,2
  1. 1Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  2. 2Marie-Josée and Henry R. Kravis Center for Molecular Oncology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  1. Correspondence to Dr Rajmohan Murali, Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; MuraliR{at}mskcc.org

Abstract

Perivascular epithelioid tumours (PEComas) of the gynaecological tract are rare tumours which were first recognised and diagnosed within the last 20 years. They represent a unique diagnostic challenge with regard to their accurate and reproducible distinction from more common entities such as smooth muscle tumours of the uterine corpus. In this review article, we trace the development of the concept of the PEComa tumour family, highlight what is known about extra-gynaecological tract PEComa at an immunohistochemical, molecular and therapeutic level and then present a summary of all reported cases of gynaecological tract PEComa to date. In the summary, we highlight rare subtypes of gynaecological tract PEComa and compare the performances of extant prognostic classification systems for malignancy in these tumours.

  • GYNAECOLOGICAL PATHOLOGY
  • NEOPLASMS
  • HISTOPATHOLOGY
  • DIAGNOSIS
  • UTERUS

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