Integrated Management of Malignant Head and Neck Paragangliomas: A Case Report and Review of Literature

Introduction

Paragangliomas are infrequent neuroendocrine neoplasms that typically arise from autonomic ganglia, predominantly presenting in the head and neck regions, with the potential for benign or malignant progression. The transformation into a malignant state poses significant diagnostic and therapeutic dilemmas, underscoring the intricacies of intervention protocols. Given the paucity of standardized treatments for advanced metastatic presentations, this case report aims to augment the comprehension of integrated treatment modalities in the management of intricate cases.

Methods

A comprehensive literature review was undertaken to evaluate the epidemiology, demographic characteristics, functional attributes, tumor localization, and genomic correlations of malignant head and neck paragangliomas, including the incidence of gene mutations associated with metastatic progression, in contrast with the parameters of the presented case.

Results

A 32-year-old man diagnosed with a paraganglioma affecting the carotid artery and showing distant metastases underwent high-risk surgery and radionuclide therapy, with a positive outcome of no cranial nerve deficits post-treatment. A review of the literature on similar cases revealed a higher occurrence in females and a general pattern of low functional capacity associated with these tumors. The dominating primary therapy for these tumors based on the literature review was surgical excision.

Conclusions

Malignant paragangliomas of the head and neck present diagnostic and treatment challenges especially given the context of advanced and distant metastasis. A multimodal approach may be required in the management of complex cases including surgery, chemotherapy, and or/radiotherapy.