Utpatel, K. and Calvisi, D. F. and Koehler, G. and Kuehnel, T. and Niesel, A. and Verioh, N. and Vogelhuber, M. and Neu, R. and Hosten, N. and Schildhaus, H-U and Dietmaier, W. and Evert, M. (2019) Complexity of PEComas. Diagnostic approach, molecular background, clinical management (German version). PATHOLOGE, 40 (4). pp. 443-453. ISSN 0172-8113, 1432-1963
Full text not available from this repository. (Request a copy)Abstract
Perivascular epithelioid cell neoplasms (PEComas) are afamily of mesenchymal neoplasms with features of both melanotic and smooth muscle differentiation. PEComa morphology is highly variable and encompasses epithelioid to spindle cells often with clear cytoplasm and prominent nucleoli. Molecularly, most PEComas are defined by aloss of function of the TSC1/TSC2 complex. Additionally, adistinct small subset of PEComas harboring rearrangements of the TFE3 (Xp11) gene locus has been identified. By presenting aseries of three case reports with distinct features, we demonstrate diagnostic pitfalls as well as the importance of molecular work-up of PEComas because of important therapeutic consequences.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ENDOMETRIAL STROMAL SARCOMAS; EPITHELIOID CELL TUMORS; MTOR INHIBITION; GENE FUSION; TFE3; ACTIVATION; NEOPLASMS; Genetic translocation; Immunohistochemistry; Lymphangioleiomyomatosis; Perivascular epithelioid cell neoplasms; TOR serine-threonine kinases |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Hals-Nasen-Ohren-Heilkunde Medicine > Abteilung für Thoraxchirurgie Medicine > Lehrstuhl für Innere Medizin III (Hämatologie und Internistische Onkologie) Medicine > Lehrstuhl für Pathologie Medicine > Lehrstuhl für Röntgendiagnostik |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 06 Apr 2020 05:34 |
| Last Modified: | 06 Apr 2020 05:34 |
| URI: | https://pred.uni-regensburg.de/id/eprint/26753 |
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