To report a single-centre experience in the endoscopic carbon dioxide laser-assisted approach to glomus tympanicum tumours.

A retrospective case review was conducted of patients diagnosed with class A1 to B1 glomus tympanicum tumours who underwent exclusive transcanal endoscopic carbon dioxide laser surgery.

Seven patients fulfilled the inclusion criteria. All patients (100 per cent) were women, with a mean age of 65.4 years (standard deviation, 13.6). There were five A2 tumours, one A1 tumour and one B1 tumour. One patient presented with a delayed tympanic membrane perforation needing myringoplasty on follow up. There were no substantial post-operative complications. The mean hospitalisation time was 9.5 hours (standard deviation, 9.8). The mean follow-up period was 32.7 months (standard deviation, 13.1), with all cases having resolution of pulsatile tinnitus and no tumour recurrence.

The study provides further evidence on the safety and efficacy of endoscopic carbon dioxide laser surgery as a minimally invasive technique for treating early-stage glomus tympanicum tumours.

This prospective case series aimed to present the outcomes of immediate selective laryngeal reinnervation.

Two middle-aged women with vagal paraganglioma undergoing an excision operation underwent immediate selective laryngeal reinnervation using the phrenic nerve and ansa cervicalis as the donor nerve. Multidimensional outcome measures were employed pre-operatively, and at 1, 6 and 12 months post-operatively.

The voice handicap index-10 score improved from 23 (patient 1) and 18 (patient 2) at 1 month post-operation, to 5 (patient 1) and 1 (patient 2) at 12 months. The Eating Assessment Tool 10 score improved from 20 (patient 1) and 24 (patient 2) at 1 month post-operation, to 3 (patient 1) and 1 (patient 2) at 12 months. There was slight vocal fold abduction observed in patient one and no obvious abduction in patient two.

Selective reinnervation is safe to perform following vagal paraganglioma excision conducted on the same side. Voice and swallowing improvements were demonstrated, but no significant vocal fold abduction was achieved.

This review summarises the contemporary, multidisciplinary approach to managing parapharyngeal space neoplasms.

Parapharyngeal space neoplasms are uncommon head and neck tumours and are most often benign. Most tumours are of either salivary gland or neurogenic origin. Patients tend to be asymptomatic even when tumours reach large sizes. Patients may present with a mass in the pharynx or neck, although frequently the tumour is found incidentally on an imaging study. Due to the limitations of physical examination in this anatomical area, imaging studies are essential to the evaluation of parapharyngeal space neoplasms. Cytopathology may provide additional diagnostic information. Open biopsy is rarely necessary and can be hazardous. Treatment is primarily surgical, and various surgical approaches can be tailored for a given neoplasm. Recently, a trend toward observation of select patients with asymptomatic neurogenic tumours has been advocated.

The evaluation and management of parapharyngeal space tumours is best done by a multidisciplinary team. Treatment should be individualised, and the risks and benefits of surgical intervention need to be carefully weighed. Complications are best avoided by careful surgical planning.

To increase awareness of the presentation, diagnostic difficulties and management of endolymphatic sac tumours.

A 79-year-old man with a 6-month history of unilateral hearing loss, tinnitus and vertigo, who was suspected to have an endolymphatic sac tumour on imaging, underwent successful transmastoid-translabyrinthine resection. A 53-year-old man with unilateral hearing loss and pulsatile tinnitus underwent subtotal resection of a suspected paraganglioma, which was identified histologically. Due to interval growth, gamma knife radiosurgery was performed followed by subtotal petrosectomy, at which juncture an endolymphatic sac tumour was reported.

A review of the world literature was carried out using Medline, which identified less than 150 reported cases of endolymphatic sac tumour.

Endolymphatic sac tumours are rare lesions of the petrous temporal bone. Although benign, they can be locally destructive. At present, there is no consensus regarding the management and long-term follow up of these tumours. Surgical resection is usually favoured, although treatment with radiotherapy and gamma knife surgery has also been reported.

We present the first published description of a painful paraganglioma of the external auditory canal. Atypical histopathology made the diagnosis difficult. We discuss the potential pitfalls of clinical diagnosis and treatment of such a case.

A 49-year-old woman presented with left-sided otalgia, hearing loss and tinnitus. Physical examination revealed a firm swelling arising from the superior portion of the left external auditory canal. A clinical diagnosis of otitis externa was made.

There was minimal response to medical treatment. The swelling was aspirated, leading to brisk bleeding. A tumour was suspected from the computed tomography scan, and confirmed by a biopsy. The patient underwent excision of the paraganglioma. The histopathology was atypical, making diagnosis difficult.

Such unusual masses of the external ear should always be borne in mind, especially when dealing with atypical presentations of commonly encountered diseases. Clinicians should have a low threshold for early intervention with imaging and biopsy.

We present the first reported case of a paraganglioma of the larynx occurring concurrently with squamous cell carcinoma in an irradiated neck.

We present a case report and a literature review of paraganglioma of the larynx.

A 78-year-old woman was found to have a mass on the laryngeal surface of the epiglottis and non-functioning larynx, four months after radical radiotherapy for biopsy-proven squamous cell carcinoma. Repeated positron emission tomography computed tomography was highly suggestive of residual malignancy. Given these findings, and the fact that the patient was aspirating, she was treated with total laryngectomy, and was found to have a paraganglioma of the larynx.

This is the first report of such a case. Its importance is three-fold in that it demonstrates: the coexistence of paraganglioma and squamous cell carcinoma; the difficulties that can be encountered in the diagnosis; and the use of radiotherapy in the treatment of paraganglioma.

We report a patient with a malignant carotid body paraganglioma treated with surgery and adjuvant radiotherapy. We discuss her treatment and outcome in the light of the published literature.

A 26-year-old woman presented with a 12-month history of a painless, left-sided neck lump. Ultrasound, computed tomography and magnetic resonance imaging revealed a carotid body tumour, which at surgical excision was found to be adherent to the vagus and hypoglossal cranial nerves (X and XII). The tumour was resected from the surrounding structures. Two local lymph nodes were removed to allow access. The internal carotid artery was also involved and had to be repaired with a synthetic graft. Histology and immunohistochemistry confirmed malignant carotid body paraganglioma. There were positive resection margins, and cervical lymph node metastasis was reported in one of the two nodes. Post-operatively, she had left Horner's syndrome, left vocal fold palsy and right upper limb weakness, all of which resolved spontaneously. She underwent adjuvant radiotherapy and remained recurrence free after 30 months.

Malignant carotid body paraganglioma can affect young adults, with an insidious onset of symptoms. In this patient, local excision (without neck dissection) and adjuvant radiotherapy were well tolerated and resulted in satisfactory local disease control.

Paragangliomas are rare tumours arising from the paraganglia of the autonomic nervous system.

We present a case of a paraganglioma arising from the hypoglossal nerve and producing an unusual clinical picture at presentation.

We supply radiological evidence of a paraganglioma originating from the hypoglossal nerve, and thus extend the evidence base for this rare site of origin. Our patient presented as an emergency with long tract neurological symptoms and progressive brainstem involvement. This presentation is not characteristic of paragangliomas in general, which usually have an indolent growth pattern and often demonstrate benign symptoms for a number of years prior to diagnosis. The location of a hypoglossal paraganglioma differs significantly from more common paragangliomas described in the neck and skull base, and this should inform the surgical approach undertaken.

A reliable diagnosis of malignant carotid body tumour can only be made in the presence of metastatic disease, because the histological features of the primary tumour do not correlate with clinical behaviour.

We report two cases of malignant carotid body tumour in which regional nodal biopsy at the time of excision of the primary tumour revealed unsuspected metastatic disease.

Reoperation in the neck for recurrent metastatic carotid body tumour is difficult and potentially hazardous. The presence of occult metastatic disease is easily identified if a selective – or sentinel – nodal dissection is performed routinely in cases of carotid body tumour excision. Such an approach adds very little morbidity, effort or time to the primary surgery, and is recommended. This view has been supported by some other authors but is generally overlooked in clinical practice.

To report a rare case of a laryngeal paraganglioma related to succinate dehydrogenase gene mutation C.

A case report and a review of the world literature concerning succinate dehydrogenase mutations and laryngeal paraganglioma are presented.

We identified a laryngeal paraganglioma in a 38-year-old woman, related to a very rare, deleterious in exon 4 of the succinate dehydrogenase mutation C. This mutation was a non-sense mutation: c.183G >A leading to p.Trp61X. No other neuroendocrine tumour was identified in this case, but a thyroid papillary carcinoma was concomitantly discovered and cured.

To our knowledge, this is the first report in the world literature of laryngeal paraganglioma related to a succinate dehydrogenase mutation C. The case presented underlines the fact that every patient with paraganglioma should be tested for succinate dehydrogenase genetic mutations, even if a family history of paraganglioma is absent, in order to enable appropriate clinical management and to improve our knowledge of familial paraganglioma.

To describe a unique presentation of a predominantly extracranial glomus faciale tumour. To discuss the role of imaging in the differential diagnosis and evaluation of a hypervascular parotid mass. To review the previous literature concerning the glomus faciale tumour.

A 54-year-old woman presented with a six-month history of facial weakness, pain and a parotid mass. Ultrasound revealed a hypervascular parotid mass and pre-operative core biopsy suggested a paraganglioma. Computed tomography defined its deep extent and demonstrated involvement of the petrous temporal bone along the descending portion of the facial nerve canal with a pattern of permeative lucency. A tumour was surgically removed which arose from the facial nerve from the second genu to the proximal divisions within the parotid gland and histology confirmed a paraganglioma.

A facial nerve glomus faciale tumour should be considered in the differential diagnosis of a hypervascular parotid mass and may present in a predominantly extracranial location. Computed tomography will prove helpful in such a case in order to limit the differential diagnosis and to define the extent of skull base involvement.