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6. Complexity of PEComas

Author

Utpatel, K., Calvisi, D. F., Köhler, G., Kühnel, T., Niesel, A., Verloh, N., Vogelhuber, M., Neu, R., Hosten, N., Schildhaus, H.-U., Dietmaier, W., and Evert, M.

Subjects
TOR serine-threonine kinases, Perivaskuläre epitheloidzellige Tumoren, congenital, hereditary, and neonatal diseases and abnormalities, Genetische Translokation, Genetic translocation, Perivascular Epithelioid Cell Neoplasms, Immunhistochemie, Lymphangioleiomyomatose, Humans, Lymphangioleiomyomatosis, Originalien, Immunohistochemistry, TOR-Serin-Threonin-Kinasen
Abstract

Perivascular epithelioid cell neoplasms (PEComas) are a family 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 a loss of function of the TSC1/TSC2 complex. Additionally, a distinct small subset of PEComas harboring rearrangements of the TFE3 (Xp11) gene locus has been identified. By presenting a series 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.

Published
2019

8. [Rare cause of necrotizing pneumonia : A case report].

Author

Gottschling M, Lerzer C, Geismann F, Schmalenberger D, Blaas S, Simsek M, Malfertheiner M, Salzberger B, Hitzenbichler F, Hamer OW, Utpatel K, Neu R, Ried M, and Mohr A

Subjects
Humans, Adult, Lung pathology, Pneumonia, Necrotizing diagnosis, Amebiasis pathology, Pneumonia diagnosis, Lung Abscess diagnosis
Abstract

A 29-year-old Indian patient was admitted to the authors' pulmonary clinic with cough and fever. Community-acquired pneumonia was initially suspected. Various antibiotic therapies were administered, which did not lead to any clinical improvement. Despite detailed diagnostics, no pathogen was found. Computed tomography showed rapidly progressive pneumonia in the left upper lobe. Since the infection could not be managed conservatively, upper lobe resection was performed. Histologically, an amoebic abscess was found to be the cause of the infection. Since cerebral and hepatic abscesses were also found, hematogenous dissemination may be assumed., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Published
2023
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