Sympathetic paraganglioma presenting with Horner's syndrome in a child

Paragangliomas of the head and neck arise from collections of cells from the neural crest associated with cranial or sympathetic nerves. Such lesions are rare in children. The majority of paragangliomas in the paediatric age groups have been familial, occurring in association with an adrenal pheochromocytoma. Children typically present with a neck mass and systemic evidence of catecholamine hypersecretion. We present the case of a 12-year-old boy with a history of increasing headache, drowsiness and Horner's syndrome for 8 months. The patient underwent exploration of the neck mass under general anaesthesia. The lesion was found to be in continuity with the left sympathetic chain. It was highly vascular but was removed en mass and sent for histological analysis. The histology showed that the mass was a well-circumscribed encapsulated tumour and that complete excision was achieved. The post-operative period was uneventful and the patient recovered well after the operation. On review at 6 months following surgery, his scars were maturing satisfactorily but the Horner's syndrome was persistent. Sympathetic chain paragangliomas are very rare tumours in the head and neck but should be considered in the differential diagnosis when clinical and radiographic evidence suggest a paraganglioma. The presentation is typically of a slow-growing neck mass with the presence of an ipsilateral Horner's syndrome. To our knowledge, this is the first reported case of a sympathetic chain paraganglioma in a child.