Giant and Rapidly Growing Malignant Peripheral Nerve Sheath Tumor of the Head and Neck with Pulmonary Metastasis: Case Report and Review of the Literature

Abstract Background：Malignant peripheral nerve sheath tumors (MPNSTs) are a subtype of malignant soft tissue sarcoma arising from peripheral nerve branches or sheaths of peripheral nerve fibers. Most cases are associated with bigger nerve stems, and are more commonly found in the proximal parts of the limbs and trunk, but rarely in the head and neck regions. However, reports on MPNSTs with pulmonary metastasis at the time of initial diagnosis are limited. Here, we present a case of MPNST that is located in the head and neck region with pulmonary metastasis and a literature review. Case description：Here, we report a case of primary malignant peripheral nerve sheath tumor of the head and neck with pulmonary metastasis. The tumor was surgically removed and fused with autogenous bone and artificial bone. The histopathological diagnosis was MPNST. However, the tumor rapidly recurred one month after surgery, and the patient exhibited progressive decline in muscle strength with numbness and an inability to move independently, so the tumor was again surgically removed. Then, she underwent adjuvant radiotherapy, chemotherapy, and antiangiogenic therapy. After three months, the patient’s tumor rapidly progressed into intracranial, neck, and lung lesions, and genetic tests were performed.Conclusions: Giant MPNSTs of the head and neck with pulmonary metastasis are highly aggressive lesions that should primarily be treated in a surgical fashion. Adjuvant radiotherapy should be initiated as soon as the patient recovers from the postoperative wound, preferably within two weeks. Antiangiogenic drugs and radiotherapy may have synergistic effects.