Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors.

@article{Wagner2010ClinicalAO,
  title={Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors.},
  author={Andrew J. Wagner and Izabela Malinowska-Kolodziej and Jeffrey A. Morgan and Wei Qin and Christopher D. M. Fletcher and Natalie Vena and Azra Hadi Ligon and Cristina R Antonescu and Nikhil Himmatsinh Ramaiya and George Daniel Demetri and David J Kwiatkowski and Robert G Maki},
  journal={Journal of clinical oncology : official journal of the American Society of Clinical Oncology},
  year={2010},
  volume={28 5},
  pages={835-40}
}
PURPOSE Perivascular epithelioid cell tumors (PEComas) represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. There is no known effective therapy for PEComa, and the molecular pathophysiology of aberrant mTOR signaling provided us with a scientific rationale to target this pathway therapeutically. On this mechanistic basis, we treated three consecutive patients with metastatic PEComa with an oral mTOR inhibitor, sirolimus. PATIENTS… CONTINUE READING
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