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Head and Neck Malignant Paragangliomas: Experience from a Single Institution
Yiming Ding1,2, Lifeng Li1,2, Demin Han1,2
1Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Ear, Nose, & Throat Journal
|October 16, 2021
Summary
Malignant paragangliomas in the head and neck, though rare, show favorable prognosis with comprehensive treatment. Genetic mutations, including in ATR and MAP3K13, are common in these tumors.
Area of Science:
- Oncology
- Genetics
- Head and Neck Surgery
Background:
- Malignant paragangliomas are rare neuroendocrine tumors affecting the head and neck.
- Understanding their clinicopathological and genetic profiles is crucial for effective management.
Purpose of the Study:
- To summarize clinicopathological and genetic features of head and neck malignant paragangliomas.
- To explore appropriate treatment strategies for this rare condition.
Main Methods:
- Retrospective review of six head and neck malignant paraganglioma cases.
- Analysis of clinicopathological characteristics, gene mutations (SDHD, SDHB, ATR, MAP3K13), and patient prognosis.
Main Results:
- Four carotid body tumors and two vagal paragangliomas were identified.
- Metastasis patterns included cervical lymph nodes, lungs, bone, and liver.
- All patients survived with a median follow-up of 66 months; genetic mutations were found in four patients.
Conclusions:
- Comprehensive treatment leads to a favorable prognosis for head and neck malignant paragangliomas.
- Genetic mutations, including novel findings in ATR and MAP3K13, are frequently observed.
