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Peer-reviewedCC BY 4.0Endocrine-Related Cancer

Tyrosine kinase inhibitors in metastatic pheochromocytomas and paragangliomas: an updated systematic review and meta-analysis

  • Xiaowen Xu1,2
  • Quanheng Liu1,2
  • Shihui Xu1
  • Awan Hasham Ikram1
  • Herve Pasteur Ndikuriyo1
  • Yizhou Wang1,2
  • Kai Cheng1
  • Zhi Li1,2
  • Junjie Chen1,2
  • Minghao Li1,2
  • Qiao Xiao3,4
  • Longfei Liu1,2
4 affiliations
  1. 1Department of Urology, Xiangya Hospital, Central South University, Changsha, China
  2. 2National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University, Changsha, China
  3. 3Department of Urology, Jiangxi Provincial People’s Hospital, Nanchang, China
  4. 4Department of Physiology, College of Medicine and Health, University College Cork, Cork, Ireland
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Abstract

Therapeutic options for metastatic pheochromocytomas and paragangliomas (mPPGLs) remain limited. Recent studies indicate that tyrosine kinase inhibitors (TKIs) offer a promising treatment approach. This study aims to evaluate the efficacy and safety of TKIs for mPPGLs based on available evidence. A systematic search for studies investigating the treatment of mPPGLs with TKIs was conducted across PubMed, Embase, and Web of Science from database inception to February 2026. Data were synthesized using fixed- or random-effects meta-analysis model. Subgroup analysis was conducted based on the status of the SDHB gene and subtypes of TKIs. A total of 11 studies, comprising 4 observational and 7 interventional studies, with 246 patients involved. The stable disease rate, objective response rate (ORR), and disease control rate (DCR) were 51.8% (95% CI: 44.6–59.1%), 27.7% (95% CI: 21.6–34.3%), and 84.2% (95% CI: 74.8–91.9%), respectively. The proportion of discontinuation due to adverse events was 11.3% (95% CI: 5.4–18.7%). The combined median progression-free survival and overall survival were 13.4 (95% CI: 7.9–14.7) and 26.7 (95% CI: 24.9–49.4) months. Pooled ORR (36.8% (95% CI: 21.1–53.8%) vs 20.1% (95% CI: 10.8–31.0%), P = 0.06) and DCR (92.2% (95% CI: 79.6–99.7%) vs 78.5% (95% CI: 57.3–94.5%); P = 0.269) were higher in patients with SDHB-related mPPGL than in those with non-SDHB mPPGL, although the differences were not statistically significant. Notably, sunitinib showed no therapeutic superiority over other TKI subtypes, with lower pooled ORR (21.2% (95% CI: 14.0–29.4%) vs 36.9% (95% CI: 26.9–47.4%); P = 0.04) and DCR (72.6% (95% CI: 63.9–80.6%) vs 94.5% (95% CI: 87.2–99.3%); P = 0.001). Consequently, TKIs show promising efficacy and manageable adverse event rates in mPPGL, supporting TKIs as components of the systematic treatment.

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Cite

BibTeXRIS
BibTeX
@article{xu2026tyrosine,
  title = {{Tyrosine kinase inhibitors in metastatic pheochromocytomas and paragangliomas: an updated systematic review and meta-analysis}},
  author = {Xiaowen Xu and Quanheng Liu and Shihui Xu and Awan Hasham Ikram and Herve Pasteur Ndikuriyo and Yizhou Wang and Kai Cheng and Zhi Li and Junjie Chen and Minghao Li and Qiao Xiao and Longfei Liu},
  journal = {Endocrine-Related Cancer},
  year = {2026},
  month = oct,
  doi = {10.1530/erc-26-0201},
  url = {https://doi.org/10.1530/erc-26-0201},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Tyrosine kinase inhibitors in metastatic pheochromocytomas and paragangliomas: an updated systematic review and meta-analysis
AU  - Xiaowen Xu
AU  - Quanheng Liu
AU  - Shihui Xu
AU  - Awan Hasham Ikram
AU  - Herve Pasteur Ndikuriyo
AU  - Yizhou Wang
AU  - Kai Cheng
AU  - Zhi Li
AU  - Junjie Chen
AU  - Minghao Li
AU  - Qiao Xiao
AU  - Longfei Liu
PY  - 2026
DA  - 2026/10/27
JO  - Endocrine-Related Cancer
DO  - 10.1530/erc-26-0201
UR  - https://doi.org/10.1530/erc-26-0201
AB  - Therapeutic options for metastatic pheochromocytomas and paragangliomas (mPPGLs) remain limited. Recent studies indicate that tyrosine kinase inhibitors (TKIs) offer a promising treatment approach. This study aims to evaluate the efficacy and safety of TKIs for mPPGLs based on available evidence. A systematic search for studies investigating the treatment of mPPGLs with TKIs was conducted across PubMed, Embase, and Web of Science from database inception to February 2026. Data were synthesized using fixed- or random-effects meta-analysis model. Subgroup analysis was conducted based on the status of the SDHB gene and subtypes of TKIs. A total of 11 studies, comprising 4 observational and 7 interventional studies, with 246 patients involved. The stable disease rate, objective response rate (ORR), and disease control rate (DCR) were 51.8% (95% CI: 44.6–59.1%), 27.7% (95% CI: 21.6–34.3%), and 84.2% (95% CI: 74.8–91.9%), respectively. The proportion of discontinuation due to adverse events was 11.3% (95% CI: 5.4–18.7%). The combined median progression-free survival and overall survival were 13.4 (95% CI: 7.9–14.7) and 26.7 (95% CI: 24.9–49.4) months. Pooled ORR (36.8% (95% CI: 21.1–53.8%) vs 20.1% (95% CI: 10.8–31.0%), P = 0.06) and DCR (92.2% (95% CI: 79.6–99.7%) vs 78.5% (95% CI: 57.3–94.5%); P = 0.269) were higher in patients with SDHB-related mPPGL than in those with non-SDHB mPPGL, although the differences were not statistically significant. Notably, sunitinib showed no therapeutic superiority over other TKI subtypes, with lower pooled ORR (21.2% (95% CI: 14.0–29.4%) vs 36.9% (95% CI: 26.9–47.4%); P = 0.04) and DCR (72.6% (95% CI: 63.9–80.6%) vs 94.5% (95% CI: 87.2–99.3%); P = 0.001). Consequently, TKIs show promising efficacy and manageable adverse event rates in mPPGL, supporting TKIs as components of the systematic treatment.
SN  - 1351-0088
SN  - 1479-6821
ER  -