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

Publikace na Lékařská fakulta v Plzni |
2021

Tento text není v aktuálním jazyce dostupný. Zobrazuje se verze "en".Abstrakt

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.