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01.12.2012 | Case report | Ausgabe 1/2012 Open Access

World Journal of Surgical Oncology 1/2012

Successful treatment with the mTOR inhibitor everolimus in a patient with Perivascular epithelioid cell tumor

Zeitschrift:
World Journal of Surgical Oncology > Ausgabe 1/2012
Autoren:
Constantine Gennatas, Vasiliki Michalaki, Paraskevi Vasilatou Kairi, Agathi Kondi-Paphiti, Dionysios Voros
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1477-7819-10-181) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

Conception and design: VM and DV. Provision of study material: CG, EKV, AKP. Collection and assembly of data: VM. Manuscript writing: VM. All authors read and approved the final manuscript.

Abstract

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm that appears to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites. Malignant PEComas exist but are very rare. These tumors represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. Metastatic PEComa is a rare form of sarcoma for which no effective therapy has been described previously and that has a uniformly fatal outcome. Although there is no known effective therapy, the molecular pathophysiology of aberrant mTOR signaling provides a scientific rationale to target this pathway therapeutically. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. On this basis, we report a case of metastatic retroperitoneal PEComa treated with an oral mTOR inhibitor, with everolimus achieving significant clinical response.
Zusatzmaterial
Authors’ original file for figure 1
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Authors’ original file for figure 2
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Authors’ original file for figure 3
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Authors’ original file for figure 4
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Literatur
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