What question did this study set out to answer?

Review the pathological classification, genetic features, diagnostic uncertainties, and therapeutic approaches for tumors arising from perivascular epithelioid cells.

December 13, 2007Virchows ArchivOpen Access

PEComas: the past, the present and the future

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Review the pathological classification, genetic features, diagnostic uncertainties, and therapeutic approaches for tumors arising from perivascular epithelioid cells.
Synthesized published clinicopathologic and cytogenetic findings regarding perivascular epithelioid cell tumors (PEComas).
Evaluated molecular signaling mechanisms linked to tuberous sclerosis complex gene disruptions and ongoing clinical trials evaluating rapamycin.
Perivascular epithelioid cells uniquely co-express melanocytic markers such as HMB45 and myogenic markers such as actin alongside recurrent chromosomal abnormalities.
Tumorigenesis is frequently linked to inactivating alterations in the TSC1 or TSC2 genes, leading to abnormal activation of the Rheb/mTOR/p70S6K signaling cascade.
Therapeutic trials targeting the mTOR axis with rapamycin in related tuberous sclerosis complex lesions provide a biological rationale for treating aggressive and malignant PEComas.

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Implication

Narrative review highlights molecular mechanisms and mTOR pathway alterations in PEComas, indicating potential clinical efficacy for rapamycin in malignant tumors.

Cite This Study

Martignoni et al. (2007) studied this question.

synapsesocial.com/papers/69deaad34838c5c0bab0c8ed https://doi.org/10.1007/s00428-007-0509-1

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Implication

Narrative review highlights molecular mechanisms and mTOR pathway alterations in PEComas, indicating potential clinical efficacy for rapamycin in malignant tumors.

PEComas: the past, the present and the future

Key Points

Discussion

Implication

Cite This Study

PEComas: the past, the present and the future

Key Points

Discussion

Implication

Cite This Study

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