Ebrahim Mahmoud A K, AlMutairi Mishal M, Hindi Khaled M, Bastaki Jassem M
Farwaniya Hospital, Ministry of Health, Kuwait City, Kuwait.
Zain Hospital, Ministry of Health, Kuwait City, Kuwait.
Case Rep Otolaryngol. 2018 Jun 10;2018:8768472. doi: 10.1155/2018/8768472. eCollection 2018.
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.
恶性外周神经鞘瘤（MPNST）是一种罕见的腮腺疾病，多数情况下预后较差。该疾病最常发生于1型神经纤维瘤病（NF1）背景下，但也可散发。在此，我们报告一例腮腺罕见的MPNST病例，患者既往无NF1病史。对该患者的初步检查包括实验室检查、肿物超声成像、细针穿刺抽吸活检（FNA）、计算机断层扫描（CT）以及颈部和肿物的磁共振成像（MRI），结果均与腮腺良性多形性腺瘤相符。然而，术中由于肿瘤与邻近组织粘连，解剖分离颇具挑战性。根据该疾病的临床病理特征，确诊为由弥漫性神经纤维瘤发展而来的MPNST。该患者表现出与（NF1）相符的临床特征，术后接受了放疗以继续治疗。
