The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
PEComas: the past, the present and the future
Open accessAbstract
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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Northrup, H., Krueger, D. A., Northrup, H., Krueger, D. A., Roberds, S., Smith, K., Sampson, J., Korf, B., Kwiatkowski, D. J., Mowat, D., et al. (2013) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2013.08.001 · Cited by 1,502
Regulation of TFEB and V-ATPases by mTORC1
Peña-Llopis, S., Vega-Rubin-de-Celis, S., Schwartz, J. C., Wolff, N. C., Tran, T. A. T., Zou, L., Xie, X.-J., Corey, D. R., & Brugarolas, J. (2011) · The EMBO Journal · doi:10.1038/emboj.2011.257 · Cited by 462
Jinzaki, M., Silverman, S. G., Akita, H., Nagashima, Y., Mikami, S., & Oya, M. (2014) · Abdominal Imaging · doi:10.1007/s00261-014-0083-3 · Cited by 332
Regulation of YAP by mTOR and autophagy reveals a therapeutic target of tuberous sclerosis complex
Liang, N., Zhang, C., Dill, P., Panasyuk, G., Pion, D., Koka, V., Gallazzini, M., Olson, E. N., Lam, H., Henske, E. P., et al. (2014) · Journal of Experimental Medicine · doi:10.1084/jem.20140341 · Cited by 205
Self-citationMolecular Therapies for Tuberous Sclerosis and Neurofibromatosis
Franz, D. N., & Weiss, B. D. (2012) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-012-0269-4 · Cited by 58
Metadata
- Authors
- Martignoni, G., Pea, M., Reghellin, D., Zamboni, G., & Bonetti, F.
- Venue
- Virchows Archiv
- Published
- 2007-12-14
- Volume
- 452
- Issue
- 2
- Pages
- 119-132
- ISSN
- 0945-6317 (ISSN-L)
- 1432-2307
- OpenAlex
- W2169304299
- Cited by
- 551
- References
- 1
- FWCI
- 12.46
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Martignoni, G., Pea, M., Reghellin, D., Zamboni, G., & Bonetti, F. (2008). PEComas: the past, the present and the future. Virchows Archiv. https://doi.org/10.1007/s00428-007-0509-1
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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