Malignant peripheral nerve sheath tumour of the parapharyngeal space in a patient with neurofibromatosis type 1.
A malignant peripheral nerve sheath tumour, the sarcoma most common in patients with neurofibromatosis type 1, arising in the parapharyngeal space is rare. They usually arise from pre-existing neurofibromas, particularly the plexiform type. The most common presenting symptoms are an enlarging neck mass, pain, and a neurological deficit. They are aggressive tumours that carry a poor prognosis despite wide excision and adjuvant radiochemotherapy. Patients with neurofibromatosis type 1 should be investigated, including deep biopsy, if pain and enlargement of the neurofibromas or any neurological deficit develop.
Sabesan
T
T
Department of Maxillofacial Surgery, Poole Hospital, Poole, Dorset BH15 2JB, UK.
Hussein
K
K
Ilankovan
V
V
D000328:Adult; D044963:Biopsy, Fine-Needle; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010610:Pharyngeal Neoplasms / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed; D018084:Ultrasonography, Interventional