title: Metastatic perivascular epithelioid cell tumor responding to mammalian target of rapamycin inhibition.
abstract: Perivascular epithelioid cell tumors (PEComa) are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Female genital tract and retroperitoneum are common sites of origin of PEComa-not otherwise specified. Diagnosis depends upon characteristic morphology and immunohistochemistry findings. Prognosis of unresectable or metastatic disease is poor. Responses to mammalian target of rapamycin (mTOR) inhibition are encouraging but mostly short-lived. We report a case of metastatic PEComa who responded to mTOR inhibition, albeit for a short duration. We also review the existing literature on mTOR inhibitors in PEComa.
authors.affiliation: Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India.
authors.forename: Indranil
authors.initials: I
authors.lastname: Ghosh
authors.affiliation: Department of Pathology, Tata Medical Center, Kolkata, West Bengal, India.
authors.forename: Indu
authors.initials: I
authors.lastname: Arun
authors.affiliation: Department of Radiodiagnosis, Tata Medical Center, Kolkata, West Bengal, India.
authors.forename: Saugata
authors.initials: S
authors.lastname: Sen
authors.affiliation: Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India.
authors.forename: Lopamudra
authors.initials: L
authors.lastname: Mishra
keywords: Immunohistochemistry; mammalian target of rapamycin inhibitors; perivascular epithelioid cell tumors; temsirolimus