Summary

A 39-year-old man was referred to the vascular surgeons with a right-sided cervical mass, palpitations, headaches and sweating. He had presented with abdominal discomfort 12 months earlier. Investigations had revealed a gastrointestinal stromal tumour (GIST) and left adrenalectomy. CT of the neck with contrast demonstrated a large right carotid paraganglioma, extending superiorly from below the carotid bifurcation to encase the internal carotid artery. Genetic screening confirmed the diagnosis of Carney-Stratakis syndrome, an SDH-D germline mutation leading to GIST and multifocal paragangliomas.

Successful surgical excision required considerable multidisciplinary teamwork between neuroendocrinologists, anaesthetists and surgeons. The tumour was highly vascular and involved the right carotid body, hypoglossal and vagus nerves. Access was challenging and maxillofacial surgical expertise were required for division of the mandible. The patient made a good recovery following speech and swallowing rehabilitation.