Uterine perivascular epithelioid cell tumour presenting as a cervical mass.
Perivascular Epithelioid Cell Tumour (PEComa) also known as myelomelanocytic tumours are uncommon, recently described mesenchymal tumours that include angiomyolipoma, clear cell "sugar" tumour of the lung, lymphangioleiomyoma and tumours composed predominantly of epithelioid cell morphology. A predilection for uterus has been described. However up till now only 14 cases of uterine PEComas have been described. All of these were seen in adult females in the peri and post menopausal age group (from 40-75years), and almost all were located in the region of body of uterus. A single case in the upper cervical region has been reported with uterine PEComatoses. We report an unusual presentation of this rare tumour presenting as a polypoidal cervical mass in a young female. Occurrence of this tumor in a young female as seen in our case warrants inclusion of PEComa in the diferential diagnosis of all epithelioid and clear cell neoplasms of uterus irrespective of age.
Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi.
Najamul Sahar
NS
Azad
Aliya Begum
AB
Aziz
Shahid
S
Pervez
Naila
N
Kayani
D000328:Adult; D000951:Antigens, Neoplasm; D002277:Carcinoma / Q000175:diagnosis* / Q000378:metabolism; D003937:Diagnosis, Differential; D015622:Epithelioid Cells / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins / Q000378:metabolism; D047648:Perimenopause; D002583:Uterine Cervical Neoplasms / Q000175:diagnosis* / Q000378:metabolism; D014594:Uterine Neoplasms / Q000175:diagnosis* / Q000378:metabolism; D014746:Vimentin / Q000378:metabolism
D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D014746:Vimentin