Successful treatment of a rare metastatic malignant carotid body tumour in a young adult, with conservative surgery and local radiotherapy

We report a patient with a malignant carotid body paraganglioma treated with surgery and adjuvant radiotherapy. We discuss her treatment and outcome in the light of the published literature. A 26-year-old woman presented with a 12-month history of a painless, left-sided neck lump. Ultrasound, computed tomography and magnetic resonance imaging revealed a carotid body tumour, which at surgical excision was found to be adherent to the vagus and hypoglossal cranial nerves (X and XII). The tumour was resected from the surrounding structures. Two local lymph nodes were removed to allow access. The internal carotid artery was also involved and had to be repaired with a synthetic graft. Histology and immunohistochemistry confirmed malignant carotid body paraganglioma. There were positive resection margins, and cervical lymph node metastasis was reported in one of the two nodes. Post-operatively, she had left Horner's syndrome, left vocal fold palsy and right upper limb weakness, all of which resolved spontaneously. She underwent adjuvant radiotherapy and remained recurrence free after 30 months. Malignant carotid body paraganglioma can affect young adults, with an insidious onset of symptoms. In this patient, local excision (without neck dissection) and adjuvant radiotherapy were well tolerated and resulted in satisfactory local disease control.