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Head and Neck Paragangliomas: Overview of Institutional Experience
Swar N Vimawala1,2, Alex Z Graboyes2, Bonita Bennett3
1Division of Otolaryngology-Head and Neck Surgery, Cooper University Health Care, Camden, NJ 08103, USA.
Cancers
|April 27, 2024
Summary
Head and neck paragangliomas (HNPGLs) are rare tumors with frequent genetic mutations. Genetic testing and metabolic evaluation are crucial for managing HNPGLs, alongside a team-based approach for better patient outcomes.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors with a high prevalence of genetic mutations.
- Understanding the genetic landscape and clinical characteristics of HNPGLs is essential for effective management.
Purpose of the Study:
- To review a single-center's experience with HNPGLs, focusing on genetic mutations, clinical presentation, and treatment outcomes.
- To emphasize the importance of genetic testing, metabolic evaluation, and multidisciplinary care for HNPGL patients.
Main Methods:
- Retrospective review of 187 patients with 296 HNPGLs diagnosed between 1974 and 2023.
- Analysis of patient demographics, genetic testing results (SDHB, SDHD mutations), metastasis/recurrence rates, metabolic findings, imaging modalities, and treatment strategies.
Main Results:
- 58.8% of tested patients had mutations, with SDHB and SDHD being most common.
- Higher metastasis and recurrence rates were observed in patients with SDHB or SDHD mutations linked to multiple PGLs.
- Elevated plasma dopamine levels were the most frequent metabolic derangement; surgery was the primary treatment (81.5%).
Conclusions:
- Genetic testing and metabolic evaluation are vital for HNPGL diagnosis and management.
- A team-based approach is recommended to improve clinical outcomes for patients with HNPGLs.
- Early identification of genetic predispositions can guide personalized treatment strategies and surveillance.
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