Purpose <p>Succinate dehydrogenase (<i>SDH</i>) mutations are strongly associated with head and neck paraganglioma (HN-PGL), with recent evidence suggesting differences in tumor behavior relative to sporadic lesions. However, the optimal treatment strategy for this population remains unclear. We therefore compared the efficacy of surgical resection versus stereotactic radiosurgery (SRS) in patients with <i>SDH-</i>mutant HN-PGL.</p> Methods <p>We retrospectively analyzed patients diagnosed with <i>SDH</i>-related HN-PGL who received treatment at our institution between September 1994 and September 2024. We reviewed clinical histories, genetic results, treatment parameters, and outcomes. Follow-up included clinical evaluations and radiological assessment. The primary outcome was local control, while the secondary outcome was treatment-related adverse events. A <i>P</i>-value of &lt; 0.05 was considered statistically significant.</p> Results <p>The study included a total of 29 patients with <i>SDH</i>-related HN-PGL, encompassing 40 lesions treated with either SRS (<i>n</i> = 16) or surgical resection (<i>n</i> = 24). Mean age at diagnosis was similar between groups (35.6 vs. 33.6 years). The majority of patients had <i>SDHD</i> mutations (75.8%), and 34.5% were symptomatic before treatment, with pulsatile tinnitus (24.1%), hearing loss (13.8%), and dysphagia (13.8%) being the most common symptoms. The median prescribed dose for SRS was 20.5&#xa0;Gy, with a median biologically effective dose of 132 Gy<sub>2</sub>. Median follow-up duration was 37.4 months and 111.0 months for SRS and resection, respectively (<i>P</i> = 0.066). SRS had significantly fewer post-treatment complications (10% vs. 47.4%, <i>P</i> = 0.046). Specifically, new or worsened hoarseness, dysphagia, and hearing loss were more prevalent in the resection group. At last follow-up, the local control rate was more favorable in the SRS versus the resection arm, although this did not rise to statistical significance in time-to-event analysis (<i>P</i> = 0.12, log-rank test). Five patients who underwent resection required reoperation.</p> Conclusion <p>SRS was associated with significantly fewer complications and a trend toward higher local control compared to surgical resection for <i>SDH</i>-related HN-PGL. Given the slow growth and frequent multifocality of hereditary HN-PGLs, a less aggressive treatment approach may reduce the need for surgery and its complications. However, further investigation with long-term follow-up is warranted to fully assess durability of disease control.</p>

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Surgical resection versus stereotactic radiosurgery for succinate dehydrogenase-related hereditary head and neck paragangliomas

  • Paul M. Harary,
  • Yusuke S. Hori,
  • Fred C. Lam,
  • Deya Abu-Reesh,
  • Sara C. Emrich,
  • Armine Tayag,
  • Louisa Ustrzynski,
  • Jennifer Alyono,
  • F. Christopher Holsinger,
  • Robert L. Dodd,
  • Iris C. Gibbs,
  • Gordon Li,
  • David J. Park,
  • Steven D. Chang

摘要

Purpose

Succinate dehydrogenase (SDH) mutations are strongly associated with head and neck paraganglioma (HN-PGL), with recent evidence suggesting differences in tumor behavior relative to sporadic lesions. However, the optimal treatment strategy for this population remains unclear. We therefore compared the efficacy of surgical resection versus stereotactic radiosurgery (SRS) in patients with SDH-mutant HN-PGL.

Methods

We retrospectively analyzed patients diagnosed with SDH-related HN-PGL who received treatment at our institution between September 1994 and September 2024. We reviewed clinical histories, genetic results, treatment parameters, and outcomes. Follow-up included clinical evaluations and radiological assessment. The primary outcome was local control, while the secondary outcome was treatment-related adverse events. A P-value of < 0.05 was considered statistically significant.

Results

The study included a total of 29 patients with SDH-related HN-PGL, encompassing 40 lesions treated with either SRS (n = 16) or surgical resection (n = 24). Mean age at diagnosis was similar between groups (35.6 vs. 33.6 years). The majority of patients had SDHD mutations (75.8%), and 34.5% were symptomatic before treatment, with pulsatile tinnitus (24.1%), hearing loss (13.8%), and dysphagia (13.8%) being the most common symptoms. The median prescribed dose for SRS was 20.5 Gy, with a median biologically effective dose of 132 Gy2. Median follow-up duration was 37.4 months and 111.0 months for SRS and resection, respectively (P = 0.066). SRS had significantly fewer post-treatment complications (10% vs. 47.4%, P = 0.046). Specifically, new or worsened hoarseness, dysphagia, and hearing loss were more prevalent in the resection group. At last follow-up, the local control rate was more favorable in the SRS versus the resection arm, although this did not rise to statistical significance in time-to-event analysis (P = 0.12, log-rank test). Five patients who underwent resection required reoperation.

Conclusion

SRS was associated with significantly fewer complications and a trend toward higher local control compared to surgical resection for SDH-related HN-PGL. Given the slow growth and frequent multifocality of hereditary HN-PGLs, a less aggressive treatment approach may reduce the need for surgery and its complications. However, further investigation with long-term follow-up is warranted to fully assess durability of disease control.