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Case Rep Otolaryngol. 2018 Jun 10;2018:8768472. doi: 10.1155/2018/8768472. eCollection 2018.

Diffuse Neurofibroma in a Patient with Unknown History of NF1.

Author information

1
Farwaniya Hospital, Ministry of Health, Kuwait City, Kuwait.
2
Zain Hospital, Ministry of Health, Kuwait City, Kuwait.
3
Kuwait Institute Medical Specialties, Kuwait City, Kuwait.
4
Al-Sabah Hospital, Ministry of Health, Kuwait City, Kuwait.

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare disease in the parotid gland with a poor prognosis in most cases. The disease most often develops in the setting of neurofibromatosis type 1 (NF1) but can also occur sporadically. Herein, we report a rare case of MPNST in the parotid gland, in a patient with no previous history of NF1. Initial investigations of the patient, which consisted of laboratory investigations, ultrasound imaging of the swelling, fine-needle aspiration (FNA), computed tomography (CT) scan, and magnetic resonance imaging (MRI) of the neck and swelling, were compatible with a benign pleomorphic adenoma of the parotid gland. However, intraoperatively, the dissection was challenging as the tumor was adherent to the neighboring tissue. A diagnosis of MPNST arising from a diffuse neurofibroma was established based on clinicopathologic features of the disease. The patient, who exhibited clinical features compatible with (NF1), proceeded for radiotherapy following surgery to continue his treatment.

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