PEComas: the past, the present and the future View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2008-02

AUTHORS

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

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. More... »

PAGES

119-132

References to SciGraph publications

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  • 2002-01. CORRESPONDENCE RE: BONETTI F, MARTIGNONI G, COLATO C, MANFRIN E, GAMBACORTA M, FALERI M, ET AL. ABDOMINOPELVIC SARCOMA OF PERIVASCULAR EPITHELIOID CELLS. REPORT OF FOUR CASES IN YOUNG WOMEN, ONE WITH TUBEROUS SCLEROSIS. MOD PATHOL 2001;14:563–8. AND TAZELAAR HD, BATTS KP, SRIGLEY JR. PRIMARY EXTRAPULMONARY SUGAR TUMOR (PEST): A REPORT OF FOUR CASES. MOD PATHOL 2001;14:615–22 in MODERN PATHOLOGY
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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s00428-007-0509-1

    DOI

    http://dx.doi.org/10.1007/s00428-007-0509-1

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1049453595

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/18080139


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