Rebekah Chetcuti, Bernard Galea
Ganglioneuromas are uncommon benign tumours derived from neural crest cells and composed of mature ganglion and Schwannian stromal elements. They most frequently arise in the posterior mediastinum and retroperitoneum, while cervical involvement is rare. Although ganglioneuroma may occur in association with neurofibromatosis type 1, such presentations are infrequently reported. Surgical excision is generally curative; however, postoperative sympathetic dysfunction, including Horner syndrome, may occur because of the close anatomical relationship of these tumours to the cervical sympathetic chain. We report the case of a 24-year-old woman with sporadic neurofibromatosis type 1 who presented with right-sided neck pain and temporomandibular discomfort. Clinical examination revealed a firm, non-tender, non-pulsatile mass in the right anterior cervical triangle, with preserved vocal cord mobility on flexible nasoendoscopy. Magnetic resonance imaging demonstrated a well-circumscribed fusiform lesion within the right carotid space extending from C1 to C6, with anterolateral displacement of the common carotid artery and splaying of the carotid bifurcation, consistent with a neurogenic tumour. Following multidisciplinary discussion, the lesion was excised via a transcervical approach. Histopathological examination confirmed ganglioneuroma with degenerative changes and no malignant features. Postoperatively, the patient developed ipsilateral ptosis and miosis consistent with Horner syndrome. Ophthalmological assessment confirmed sympathetic dysfunction. Her symptoms gradually resolved, with complete recovery by six months. Follow-up magnetic resonance imaging demonstrated no residual or recurrent disease, and serial imaging over four years showed no recurrence and stability of a smaller contralateral lesion. This case highlights cervical ganglioneuroma as a rare differential diagnosis for carotid space masses in patients with neurofibromatosis type 1. Although complete excision offers an excellent prognosis, careful preoperative counselling and operative planning are essential because of the risk of temporary sympathetic chain dysfunction, particularly in lesions extending toward the skull base.