Glomus tumours of the skull base are rare, and most frequently present with symptoms of hearing loss and tinnitus. Diagnosis is often delayed due to the slow growth of the tumour. We describe the previously unreported occurrence of a glomus tumour presenting with a unilateral accessory nerve palsy.

This review describes the features of glomus jugulare tumours with metastases. There were 100 sites of metastasis in the 53 cases previously reported. The sites of metastasis may be summarized as bone (33), lungs (23), lymph nodes (19), liver (nine) and other (16). Metastases presented up to 30 years after the initial treatment. The mean age of patients was 45 years and the sex ratio was approximately two females to one male, with no significant difference compared to non-metastatic tumours. There was a significantly higher incidence of pain and a significantly lower incidence of hearing loss at presentation compared to non-metastatic tumours. The commonest treatment was a combination of surgery and radiotherapy. The duration of symptoms before diagnosis was significantly shorter and the rates of persistent or recurrent local disease and death were significantly higher than for non-metastatic tumours. This review highlights the fact that glomus jugulare tumours are not always benign.

Glomus jugulare tumours are classically described as benign tumours with a long time course often measured in decades. Although these tumours may be locally invasive, most cases are histologically benign and metastases are rare. The case of a malignant glomus jugulare tumour with a particularly aggressive pattern of spread is presented. At the time of surgery, which was within 12 months of the development of symptoms, intracranial spread and metastasis to cervical lymph nodes had already occurred, demonstrating that glomus jugulare tumours are not always benign.

Two cases of metastatic carcinoma of the temporal bone, that simulated glomus tumours on thorough preoperative evaluation are described. Although rare, metastatic spread to this area is recognized, but presentation in this way is unique.

The ISG viewing wand is an intra-operative guidance system with a proprioceptive robotic-like jointed arm. It provides surgeons with almost instantaneously reconstructed computer-generated CT or MRI images in two or three dimensions and can correlate any point within the operative field to its corresponding locus on the reformatted scan images. In addition to having been used in over 400 neurosurgical patients in Bristol, 14 patients with skull-base, cerebello-pontine angle or temporal bone lesions have also undergone wand-guided resections. The wand has proved to be particularly useful in pre-operative planning, allowing minimally-invasive incisions, providing per-operative navigation, identifying the relationship and proximity of important anatomical structures and in assessing the extent of lesion resection. We illustrate the advantages of intra-operative image-guidance by discussing four cases. The potential applications of this form of imaging technology to other otolaryngological procedures are discussed.

A 47-year-old man presented, in 1990, with a short history of left-sided cerebellar ataxia. In 1986 he had undergone excision of a glomus jugulare tumour. A magnetic resonance (MRI) scan demonstrated cerebellar herniation through a defect in his skull base. Surgical repair was undertaken with resolution of his symptoms.