Giant Malignant Peripheral Nerve Sheath Tumor of the Head and Neck: A Case Report and Literature Review

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a malignant soft tissue sarcoma with high mortality, low morbidity, and poor prognosis. The MPNST occurs mostly in the limbs and torso, and rarely in the head and neck. However, MPNST is insensitive to radiotherapy and chemotherapy, and complete surgical resection with negative margin is the most important and effective strategy. We present a case of MPNST in the head and neck. The tumor invades the left temporal bone, petrous bone, and mastoid bone, and compression changes in the focal cerebellum and sigmoid sinus. The patient underwent the left temporal region tumor resection + surgical reconstruction with temporalis muscle flap and pectoralis major myocutaneous flap. Adjuvant radiotherapy (55 Gy) was given after surgery, and there were no local recurrence and distant metastasis after 31-month follow-up.