Head and neck paragangliomas are rare. They are usually slow-growing, benign, non-catecholamine secreting tumours, traditionally treated with surgical excision. Complications of surgical excision include lower cranial nerve palsies, stroke and death.

A retrospective case note analysis was conducted of patients with head and neck paragangliomas treated with a watch-and-scan policy from March 2003 to September 2015, and the relevant literature was reviewed.

Fifteen head and neck paragangliomas were identified. None of the patients developed a new lower cranial nerve palsy or progression of their presenting hearing loss during the follow-up period. Five patients displayed an increase in maximum linear dimension of 4 mm over an average of 57.4 months. A review of the literature showed that a watch-and-surveillance scan policy is evolving as a treatment option for head and neck paragangliomas without malignant risk factors.

Readily available surveillance scanning in head and neck paragangliomas enables the monitoring of head and neck paragangliomas, which may allow for avoidance of major surgery.

To provide an update on the ever-increasing role that embolisation plays in the practice of otolaryngology.

A literature search was performed during November 2008. The Medline, Embase, PubMed and Cochrane databases were searched. This resulted in 285 papers relevant for review.

The role of embolisation has expanded greatly to include the management of refractory epistaxis, pre-operative preparation of vascular tumours, vascular injuries and as an adjunct in skull base surgery.

We report the case of a recurrent familial malignant carotid body tumour presenting with metastasis to local ipsilateral lymph nodes; the rarity of both recurrence combined with nodal spread is emphasised in this article.

We present a case report, and a review of the world literature concerning the diagnosis and management of carotid body tumours in the familial setting.

A woman with a family history of succinate dehydrogenase complex subunit B gene mutation presented with right vocal fold palsy. A causative carotid body tumour was excised. Fifteen years later, the patient developed a right-sided swelling in the jugulo-digastric region, together with shooting pains towards her right ear. Imaging revealed right posterior triangle lymphadenopathy. Fine needle aspiration cytology of the node was equivocal. Computed tomography of her neck revealed, in addition, a mass within the right side of the larynx. Excision biopsy of the lymph node demonstrated metastatic paraganglioma. A carotid angiogram revealed a right-sided carotid body tumour. This was embolised prior to neck exploration and excision of the carotid body tumour with en bloc resection of adjacent nodes. Histological analysis confirmed the presence of lymph nodes containing metastatic paraganglioma.

This case report demonstrates the need for extra vigilance to enable early disease detection in the familial setting of carotid body tumour, in order to reduce the surgical morbidity associated with disease progression. In addition, our report highlights the atypical aspects of presentation in the familial setting, together with the difficulty and lack of standardisation regarding monitoring of the disease.

Carotid body tumours are rare lesions which are familial in 10 per cent of cases. In this paper, we demonstrate the clinical applicability of predictive genetic testing for familial carotid body tumours.

We report a case manifesting with multiple carotid body tumours, in which subsequent genetic testing demonstrated a germline mutation which could be traced across generations. We review the diagnosis and management of carotid body tumours in the familial setting, together with the strategies presently available to screen individuals from susceptible families.

The recent advent of a predictive genetic test for familial carotid body tumours offers a novel means of pre-selecting those at risk, so as to minimise screening costs and patient morbidity. Early diagnosis of lesions is essential to allow early intervention, reducing surgical morbidity and progression to malignancy.