Repetitive syncope caused by a rare massive sporadic malignant peripheral nerve sheath tumor involving carotid arteries: A case report

• Published in: Medicine (Baltimore) 2021-02, Vol.100 (5), p.e24386-e24386
• Main Authors: Wang, Tiehao, Wang, Jiarong, Zhao, Jichun, Yuan, Ding, Yao, Wenqing
• Format: Article
• Language: English
• Subjects: Aged; Carotid Arteries - pathology; Clinical Case Report; Female; Humans; Nerve Sheath Neoplasms - complications; Nerve Sheath Neoplasms - pathology; Peripheral Nervous System Neoplasms - complications; Peripheral Nervous System Neoplasms - pathology; Sarcoma - complications; Sarcoma - pathology; Syncope - etiology
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000024386

Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas arising from peripheral nerves. MPNSTs are uncommon in the head and neck, and various clinical manifestation often make the diagnosis challenging. A 67-year-old female was referred for evaluation of repetitive syncope with a massive mass in the neck. Preoperative evaluation revealed potential neuroendocrine activity of the mass and enhanced computed tomography showed carotid artery was involved. According to the preoperative imaging, intraoperative finding and postoperative pathological examination, the diagnosis of left neck MPNST involving left carotid arteries was made. Volume expansion therapy with phenoxybenzamine started one week before surgery. Complete surgical resection of the mass was performed and pathological analysis suggested the diagnosis of MPNST. The postoperative radiotherapy was not given due to her poor nutrition. This patient recovered well after surgery and no sign of recurrence was noted at 2-year follow-up. Though the involvement of carotid artery with neuroendocrine activity is rare in sporadic MPNST, preoperative scanning of blood and urine catecholamine is crucial for intraoperative hemodynamic stability, especially when carotid artery is involved.