| 論文種別 | 原著(症例報告除く) |
| 言語種別 | 英語 |
| 査読の有無 | その他(不明) |
| 表題 | Clear Cell Papillary Renal Cell Tumor Revisited: Comparison of Histologic and Immunohistochemical Profiles in Relation to VHL Allelic Status. |
| 掲載誌名 | 正式名：The American journal of surgical pathology 略 称：Am J Surg Pathol ISSNコード：15320979/01475185 |
| 掲載区分 | 国外 |
| 巻・号・頁 | pp.Online ahead of print |
| 著者・共著者 | Kanako Sagan, Fumiyoshi Kojima, Ibu Matsuzaki, Keisuke Hanada, Fidele Y Musangile, Takahiro Nakamoto, Hiroyuki Hayashi, Chisato Ohe, Riuko Ohashi, Yoji Nagashima, Yasuo Kohjimoto, Shin-Ichi Murata |
| 発行年月 | 2026/03 |
| 概要 | Clear cell papillary renal cell tumor (CCPRCT), formerly known as clear cell papillary renal cell carcinoma, is a low-grade renal neoplasm characterized by cytokeratin 7 and cup-like carbonic anhydrase 9 immunopositivity and absence of von Hippel-Lindau (VHL) abnormalities. Given the prognostic differences, distinguishing CCPRCT from clear cell renal cell carcinoma (CCRCC) is clinically important. However, some tumors diagnosed morphologically and immunohistochemically as CCPRCT have been found to harbor VHL abnormalities, which complicates clinical management. This study aimed to clarify how clinical, histologic, and immunohistochemical characteristics varied based on VHL status. Among 17 CCPRCTs analyzed, VHL abnormalities were identified in 10 (58.8%) cases, including VHL mutations in 6 cases and 3p loss of heterozygosity in 7 cases. Tumors were stratified into 3 groups based on VHL allele status: 3 cases with biallelic VHL inactivation (CCPRCT-biVHL), 7 with monoallelic VHL inactivation (CCPRCT-monoVHL), and 7 with wild-type VHL (CCPRCT-wtVHL). We integrated the molecular with morphologic and immunohistochemical findings, and reclassified 17 cases into 5 CCRCCs mimicking CCPRCT, 5 CCPRCTs-monoVHL, and 7 CCPRCTs-wtVHL cases. All tumors exhibited favorable clinical courses, with no instances of metastasis or recurrence. No significant differences in clinical, pathologic, or immunohistochemical features were observed among the 3 groups or between CCRCCs mimicking CCPRCT and CCPRCTs. This study demonstrated that strict morphologic and immunohistochemical criteria can distinguish most CCRCCs mimicking CCPRCT from CCPRCT. The diagnosis of CCPRCT should be expanded to include tumors with monoallelic VHL alteration exhibiting typical morphologic and immunohistochemical features. |
| DOI | 10.1097/PAS.0000000000002535 |
| PMID | 41797440 |