Perivascular epithelioid tumours (PEComas) of the gynaecological tract.
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.
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Niamh
N
Conlon
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Robert A
RA
Soslow
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA Marie-Josée and Henry R. Kravis Center for Molecular Oncology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Rajmohan
R
Murali
D000328:Adult; D000368:Aged; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics; D003937:Diagnosis, Differential; D005260:Female; D005833:Genital Neoplasms, Female / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000473:pathology*; D011379:Prognosis; D014178:Translocation, Genetic / Q000235:genetics
D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; C069203:TFE3 protein, human
DIAGNOSIS; GYNAECOLOGICAL PATHOLOGY; HISTOPATHOLOGY; NEOPLASMS; UTERUS