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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BMC
2025-07-01
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| Online Access: | https://doi.org/10.1186/s12885-025-14562-6 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Junjie Bai |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-a3219849ef8f44b29106b401b7a28a7a |
| institution | Kabale University |
| issn | 1471-2407 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj-art-a3219849ef8f44b29106b401b7a28a7a2025-08-20T03:42:53ZengBMCBMC Cancer1471-24072025-07-0125111410.1186/s12885-025-14562-6Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective studyJunjie Bai0Jianjia Huang1Yushi Ye2Jun Lin3Yahui Wen4Zicheng Cai5Peihuang Chen6Tong Shangguan7Gaoyu Zou8Qianyi Qiu9Ru Chen10Rong Liu11Weizhong Cai12Shaoxing Zhu13Jianhui Chen14Xiaoyan Li15Department of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalThe Graduate School of Fujian Medical UniversityDepartment of Pathology, Zhongshan Hospital, Xiamen UniversityDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Urology, Fujian Medical University Union HospitalDepartment of Pathology, Fujian Medical University Union HospitalAbstract 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.https://doi.org/10.1186/s12885-025-14562-6FH-Deficient renal cell carcinomaImmunohistochemicalProgrammed death ligand-1 (PD-L1)Programmed death ligand-2 (PD-L2) |
| spellingShingle | 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 Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study BMC Cancer FH-Deficient renal cell carcinoma Immunohistochemical Programmed death ligand-1 (PD-L1) Programmed death ligand-2 (PD-L2) |
| title | Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study |
| title_full | Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study |
| title_fullStr | Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study |
| title_full_unstemmed | Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study |
| title_short | Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study |
| title_sort | clinicopathologic and prognosis of 30 patients with fh deficient renal cell carcinoma a single center retrospective study |
| topic | FH-Deficient renal cell carcinoma Immunohistochemical Programmed death ligand-1 (PD-L1) Programmed death ligand-2 (PD-L2) |
| url | https://doi.org/10.1186/s12885-025-14562-6 |
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