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The following is a summary of “Head and Neck Paragangliomas: Recent Advances in Translational and Clinical Research and Guidelines for Patient Care,” published in the September 2024 issue of Endocrinology by Richter et al.
Head and neck paragangliomas (HNPGLs) are infrequent neuroendocrine tumors primarily from parasympathetic ganglia along the cranial nerves. Their management presents significant challenges due to several factors, including their complex anatomical origin, propensity for aggressive infiltration into neurovascular structures and the skull base, unpredictable metastatic potential, resistance to both radiotherapy and chemotherapy, and the risk of multifocality. Symptoms associated with HNPGLs can vary widely, ranging from mild discomfort to life-threatening conditions, depending on the tumor’s size and location; however, they rarely result in catecholamine excess. Key risk factors for developing HNPGLs include female sex and pathogenic germline variants in genes related to hypoxia signaling, particularly those associated with succinate dehydrogenase.
The diagnostic evaluation for HNPGLs is multifaceted, relying on advanced imaging techniques, plasma-free metanephrines and methoxytyramine measurement, genetic testing, and thorough pathology and immunohistochemistry assessments. Treatment strategies are individualized based on patient and tumor characteristics. They may include a range of approaches, such as observation with a wait-scan strategy, upfront surgical intervention, debulking procedures, and radiotherapy. Preoperative embolization is typically advised, except in the case of small tympanic and tympanomastoid tumors, while preoperative stenting is essential for managing the involvement of the internal carotid artery. In cases of intradural extension, a two-stage surgical approach may be necessary to achieve optimal outcomes. Despite these interventions, current treatment modalities for metastatic or inoperable HNPGLs remain non-curative, underscoring the importance of long-term follow-up for all patients.
Such monitoring is crucial to detect local recurrences and the emergence of new tumors, thereby facilitating timely intervention and management. Given the complexities associated with HNPGLs, an integrated multidisciplinary approach is vital in optimizing patient care, improving clinical outcomes, and advancing the understanding of the long-term implications of this challenging disease. Ongoing research into the molecular underpinnings of HNPGLs and developing novel therapeutic strategies is essential to enhance treatment efficacy and patient prognosis in this rare and intricate tumor type.
Source: sciencedirect.com/science/article/pii/S1521690X24001271
