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Review of TFEB-amplified renal cell carcinoma with focus on clinical and pathobiological aspects.

Authors:
Naoto Kuroda Emiko Sugawara Chisato Ohe Fumiyoshi Kojima Riuko Ohashi Shuji Mikami Yoji Nagashima Kvetoslava Peckova Michal Michal Ondrej Hes

Pol J Pathol 2021 ;72(3):197-199

Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzen, Pilsen, Czech Republic.

The disease entity of TFEB-amplified renal cell carcinoma (RCC) has been recently established. In this article, we review such cases. Clinically, the age of patients ranged from 28 to 83 years with a mean age of 62.8 years. The size of the tumor ranged from 1.9 to 19.5 cm with a mean size of 8.7 cm. The tumor demonstrated a variety of architectural patterns such as solid, alveolar, papillary, pseudopapillary, nested or tubular. The International Society of Urological Pathology (ISUP) grade usually corresponds to grade 3 or 4. Cytomorphology shows eosinophilic, clear, amphophilic or even oncocytic cytoplasm. Necrosis can be frequently observed. Neoplastic cells with TFEB-amplified RCC show diffuse or patchy positivity for TFEB. Fluorescence in situ hybridization frequently show the amplification of more than 10 or 20 copies of the TFEB gene. Most TFEB-amplified RCCs behave in an aggressive fashion. Metastasis frequently occurs. In conclusion, this tumor seems to be characterized by occurrence in older patients, frequent necrosis, papillary/pseudopapillary growth pattern, high-grade nuclear grade, TFEB gene amplification, and aggressive clinical behavior. In order to clarify whether this tumor is a distinct entity from previously described renal tumors or not, a further examination in a large scale study will be required in the future.

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http://dx.doi.org/10.5114/pjp.2021.111769DOI Listing
January 2022

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