PEComas: what are the treatment options? literature review and personal experience


S.A. Protsenko, G.M. Teletaeva, E.A. Degtiareva, E.V. Levchenko, N.E. Levchenko, A.V. Mishchenko, I.A. Burovik, A.S. Artemyeva, E.N. Imyanitov, A.I. Semenova, A.V. Novik, D.Kh. Latipova

N.N. Petrov National Medical Research Center of Oncology, St. Petersburg, Russia
Background. Perivascular epithelioid cell tumors (PEComas) are extremely rare mesenchymal neoplasms that can affect almost any organ. Difficulties of diagnosis, lack of prognostic factors and adequate treatment reduce the overall survival of patients with this pathology. The article presents the latest achievements in the field of drug treatment of malignant PEComas.
Description of the clinical case. An own clinical observation of successful application of everolimus in 40-year-old patient suffering from a malignant PEComa of the uterus with lung metastases is presented. Therapy lasts more than 7 months, the maximum effect of the treatment is stabilization of the process.
Conclusion. Standard chemotherapy regimens are ineffective for PEComas and are accompanied by the rapid development of process progression. Analysis of the molecular mechanisms of the disease led to the emergence of a new class of drugs - mTOR inhibitors, currently the main method of drug treatment of this pathology. Nevertheless, the issue of choosing the second and subsequent lines of therapy has not been fully studied. However, timely and correct diagnosis determines a higher efficacy of the treatment and, therefore, improves the quality of life and life expectancy of this category of patients.
Keywords: PEComa, chemotherapy, targeted therapy, mTOR inhibitors, immunotherapy, hormone therapy

About the Autors

Corresponding author: Svetlana A. Protsenko, MD, Professor, Head of the Department of Chemotherapy and Innovative Technologies, N.N. Petrov National Medical Research Center of Oncology, St. Petersburg, Russia; e-mail:
Address: 68, Leningradskaya Street, Pesochny settlement, St. Petersburg 197758, Russian Federation

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