We report a 38-year-old woman with a malignant catecholamine-secreting vagal paraganglioma. In the preceding year she had intermittent severe frontoparietal headaches. While she was receiving radiotherapy (35 Gy in 15 fractions) she developed palpitations, which steadily worsened over the following three years. A repeat CT scan showed no change in the size of the tumor. Urinary catecholamines were elevated and a MIBG scan showed increased uptake in the region of the vagal paraganglioma. She underwent near total resection of her tumor via a Fisch type C approach. Pathological examination showed a paraganglioma with metastasis involving a cervical lymph node. The palpitations and headaches resolved completely after surgery. This report and other published case reports suggest that catecholamine secretion from head and neck paragangliomas does not appear to respond to radiotherapy. Patients with cardiovascular symptoms from catecholamine-secreting paragangliomas are best managed surgically.
Paraganglioma - extra-adrenal - catecholamines - radiotherapy - surgery