Otolaryngology Case Reports (Nov 2020)
Management of a rapidly expanding neck mass with a rare diagnosis
Abstract
Malignant peripheral nerve sheath tumours are uncommon, aggressive tumours that are very rarely found in the head and neck. Whilst commonly associated with NF1 and radiotherapy, they can on occasion present sporadically. MPNSTs provide a challenge to the pathologist in diagnosis due to the often heterogeneous nature of their composition.A 60 year old man initially presented to primary care with a 2 cm neck mass, which was biopsied with fine needle aspiration, demonstrating spindle-shaped cells positive for S100 and SOX10 on immuneohistochemistry. By the time the patient presented to secondary care, the tumour had enlarged to 8 cm. Staging scans showed no evidence of metastasis. Surgery was carried out, after which histology was consistent with the diagnosis of MPNST. Histologically the tumour consisted of moderately atypical and pleomorphic spindle cells which in areas had a fascicular growth pattern. Four weeks post-operatively, a CT scan demonstrated pulmonary metastases, which progressed despite chemotherapy. The patient died 10 months after surgery.Rapid referral to secondary care of any quickly expanding neck mass must always be considered. Although extremely rare, a diagnosis of MPNST should be contemplated in the presence of a rapidly enlarging neck mass with nerve involvement, even in the absence of NF1. Histological diagnosis is key to accurate identification of MPNST, and surgery is by far the most effective treatment.
