Intraparotid neurofibroma: review of the literature

To perform a literature review of intra-parotid neurofibromas. These tumours present a challenge because of their risk of malignant transformation and their high probability of operative facial nerve compromise.

A description of one case of intraparotid neurofibroma. A systematic MEDLINE review was performed to document all reported cases.

Pediatric tertiary care centre.

An 14-month-old boy was referred with a nontender right-sided 4 × 2.5 cm preauricular mass, which was slowly increasing in size for 8 months. No facial nerve compromise was present.

During superficial parotidectomy, the mass was found to involve the facial nerve and complete resection was performed, on which a cerebrospinal fluid (CSF) leak originating from the stylomastoid foramen occurred. Mastoidectomy revealed an intramastoid division of the facial nerve. The leak coming from the facial nerve was successfully controlled with anastomosis of the major division with the distal nerves.

Histopathologic analysis revealed a diffuse neurofibroma. Twenty-two previous cases of intraparotid neurofibroma have been described, although this is the first reported case of the diffuse subtype. Preoperative fine-needle aspiration was inconclusive, and incisional biopsy was dangerous.

It seems prudent to delay surgery for asymptomatic pediatric patients with facial neurofibroma. Surgery with primary facial nerve grafting is an effective option for patients with facial function of grade III or worse or when the tumour is large, disfiguring, and aggressive. Potential complications of treatment vary depending on the location of the tumour. These include disease recurrence, facial nerve compromise, and CSF leak.