PMID- 25006296 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140709 LR - 20211021 IS - 0971-5851 (Print) IS - 0975-2129 (Electronic) IS - 0971-5851 (Linking) VI - 35 IP - 1 DP - 2014 Jan TI - Metastatic perivascular epithelioid cell tumor responding to mammalian target of rapamycin inhibition. PG - 99-102 LID - 10.4103/0971-5851.133733 [doi] AB - 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. FAU - Ghosh, Indranil AU - Ghosh I AD - Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India. FAU - Arun, Indu AU - Arun I AD - Department of Pathology, Tata Medical Center, Kolkata, West Bengal, India. FAU - Sen, Saugata AU - Sen S AD - Department of Radiodiagnosis, Tata Medical Center, Kolkata, West Bengal, India. FAU - Mishra, Lopamudra AU - Mishra L AD - Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India. LA - eng PT - Case Reports PL - Germany TA - Indian J Med Paediatr Oncol JT - Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology JID - 9604571 PMC - PMC4080675 OTO - NOTNLM OT - Immunohistochemistry OT - mammalian target of rapamycin inhibitors OT - perivascular epithelioid cell tumors OT - temsirolimus COIS- Conflict of Interest: None declared. EDAT- 2014/07/10 06:00 MHDA- 2014/07/10 06:01 PMCR- 2014/01/01 CRDT- 2014/07/10 06:00 PHST- 2014/07/10 06:00 [entrez] PHST- 2014/07/10 06:00 [pubmed] PHST- 2014/07/10 06:01 [medline] PHST- 2014/01/01 00:00 [pmc-release] AID - IJMPO-35-99 [pii] AID - 10.4103/0971-5851.133733 [doi] PST - ppublish SO - Indian J Med Paediatr Oncol. 2014 Jan;35(1):99-102. doi: 10.4103/0971-5851.133733.