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Open Access 01-02-2008 | Review and Perspective

PEComas: the past, the present and the future

Authors: Guido Martignoni, Maurizio Pea, Daniela Reghellin, Giuseppe Zamboni, Franco Bonetti

Published in: Virchows Archiv | Issue 2/2008

Abstract

The perivascular epithelioid cell (PEC) is a cell type constantly present in a group of tumors called PEComas. PEC expresses myogenic and melanocytic markers, such as HMB45 and actin. Recently, recurrent chromosomal alterations have been demonstrated in PEC. At present, PEComa is a widely accepted entity. In the past 10 years, the use of this term has allowed to report and describe numerous cases permitting to start highlighting the biology of this group of lesions. PEComas are related to the genetic alterations of tuberous sclerosis complex (TSC), an autosomal dominant genetic disease due to losses of TSC1 (9q34) or TSC2 (16p13.3) genes which seem to have a role in the regulation of the Rheb/mTOR/p70S6K pathway. There are some open questions about PEComas regarding its histogenesis, the definition of epithelioid angiomyolipoma and the identification of the histological criteria of malignancy. An innovative therapeutic trial using rapamycin is under way for tumors occurring in TSC such as renal angiomyolipoma and lymphangioleiomyomatosis. Its success could provide the rationale for the use of the same drug in other lesions composed of PECs, especially in the malignant ones.

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Title: PEComas: the past, the present and the future
Authors: Guido Martignoni
Maurizio Pea
Daniela Reghellin
Giuseppe Zamboni
Franco Bonetti
Publication date: 01-02-2008
Publisher: Springer-Verlag
Published in: Virchows Archiv / Issue 2/2008
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-007-0509-1

At a glance: The STEP trials

Springer Medicine

PEComas: the past, the present and the future

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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