REVIEW ARTICLE
Review of TFEB-amplified renal cell carcinoma with focus on clinical and pathobiological aspects
 
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1
Department of Diagnostic Pathology, Kobe Kyodo Hospital, Kobe, Japan
 
2
Department of Pathology, The Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan
 
3
Department of Pathology and Laboratory Medicine, Kansai Medical University, Hirakata, Japan
 
4
Department of Human Pathology, Wakayama Medical University, Wakayama, Japan
 
5
Histopathology Core Facility, Niigata University Faculty of Medicine, Niigata, Japan
 
6
Department of Diagnostic Pathology, Keio University School of Medicine, Tokyo, Japan
 
7
Department of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan
 
8
Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzen, Pilsen, Czech Republic
 
 
Submission date: 2020-12-28
 
 
Acceptance date: 2021-07-27
 
 
Publication date: 2022-01-19
 
 
Pol J Pathol 2021;72(3):197-199
 
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ABSTRACT
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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ISSN:1233-9687
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