Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study

Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study

Abstract Background FH-Deficient Renal Cell Carcinoma (FH-RCC) is a rare, aggressive cancer with poor prognosis and treatment challenges. Immunohistochemical (IHC), including PD-1, PD-L1, PD-L2, and CTLA-4, play a key role in survival outcomes and therapeutic decisions, highlighting the need to eval...

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Main Authors: Junjie Bai, Jianjia Huang, Yushi Ye, Jun Lin, Yahui Wen, Zicheng Cai, Peihuang Chen, Tong Shangguan, Gaoyu Zou, Qianyi Qiu, Ru Chen, Rong Liu, Weizhong Cai, Shaoxing Zhu, Jianhui Chen, Xiaoyan Li
Format: Article
Language:English
Published: BMC 2025-07-01
Series:BMC Cancer
Subjects:
FH-Deficient renal cell carcinoma
Immunohistochemical
Programmed death ligand-1 (PD-L1)
Programmed death ligand-2 (PD-L2)
Online Access:https://doi.org/10.1186/s12885-025-14562-6
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Summary:Abstract Background FH-Deficient Renal Cell Carcinoma (FH-RCC) is a rare, aggressive cancer with poor prognosis and treatment challenges. Immunohistochemical (IHC), including PD-1, PD-L1, PD-L2, and CTLA-4, play a key role in survival outcomes and therapeutic decisions, highlighting the need to evaluate their significance in FH-RCC. Methods The expression of IHC including PD-1, PD-L1, PD-L2 and CTLA-4 in tumor cells was evaluated. The clinicopathological and therapeutic information of 30 patients with FHRCC was retrospectively collected and correlation analysis and Kaplan-Meier analysis were performed. Results In a cohort of 30 patients with FHRCC, the majority of tumors exhibited a simple papillary structure. PD-1 positive expression was observed in 43.3% (13/30) of the patients, PD-L1 in 53.3% (16/30), PD-L2 in 20.0% (6/30), and CTLA-4 in 90%. CK-7 showed positive expression in 23.3% of the patients. Notably, PD-L1 positivity was associated with a higher frequency of simple papillary structures and better outcomes in patients receiving TKI monotherapy. Conversely, patients who underwent radical surgery, exhibited negative CK-7 expression, had positive PD-1 expression, and negative PD-L2 expression, demonstrated shorter disease-free survival (DFS). Additionally, those who underwent radical surgery and were PD-L2 negative had worse cancer-specific survival (CSS). Conclusion FH-RCC is highly aggressive and has a poor prognosis, and clinical and pathologic parameters such as IHC expression including PD-1, PD-L1, PD-L2 and CTLA-4 have a predictive value in prognosis and treatment.
ISSN:1471-2407

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