Paragangliomas are rare neuroendocrine tumors originating from paraganglia in sympathetic or parasympathetic ganglia. While often benign, their location—especially in the skull base, skull base, neck, or paravertebral regions—can pose significant management challenges. Paragangliomas can arise from sympathetic neural crests (principally in the extra adrenal area and abdomen) or from parasympathetic ganglia (principally in the head and neck and skull base regions). Malignant transformation has been noted in a minority of cases especially in the temporal area and carotid region. Surgery has historically been the primary treatment modality, but radiation therapy (RT) has emerged as an effective option in select cases. This chapter reviews the epidemiology, pathogenesis, indications for RT, technical considerations, toxicity profiles, and outcomes of RT in paragangliomas. Additionally, recent literature, case series, and future directions—including proton therapy and stereotactic approaches—are explored.

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Paragangliomas

  • Ashutosh Mukherji

摘要

Paragangliomas are rare neuroendocrine tumors originating from paraganglia in sympathetic or parasympathetic ganglia. While often benign, their location—especially in the skull base, skull base, neck, or paravertebral regions—can pose significant management challenges. Paragangliomas can arise from sympathetic neural crests (principally in the extra adrenal area and abdomen) or from parasympathetic ganglia (principally in the head and neck and skull base regions). Malignant transformation has been noted in a minority of cases especially in the temporal area and carotid region. Surgery has historically been the primary treatment modality, but radiation therapy (RT) has emerged as an effective option in select cases. This chapter reviews the epidemiology, pathogenesis, indications for RT, technical considerations, toxicity profiles, and outcomes of RT in paragangliomas. Additionally, recent literature, case series, and future directions—including proton therapy and stereotactic approaches—are explored.