Malignant Peripheral Nerve Sheath Tumor of the Cervix

The occurrence of primary malignant peripheral neurilemmoma in the female cervix without neurofibromatosis type 1 is extremely rare. We, herein, report a case of a 46-year woman with malignant peripheral nerve sheath tumor (MPNST) involving the cervix. The patient was admitted to the hospital because of irregular vaginal bleeding. Pelvic CT suggested the presence of cervical mass, and the patient accepted radical hysterectomy. Following the surgery, MPNST was diagnosed by a combination of histology and immunohistochemistry. One year later, the patient developed recurrence and pulmonary metastasis and received sequentially epiubicin/ifosfamide, dacarbazine, and etoposide/cisplatin multi-line chemotherapy. The prognosis of MPNST is poor, which is related to its invasiveness, high recurrence and metastasis rates. The patient was treated with surgery, multi-line chemotherapy and targeted therapy, but the overall survival (OS) was 44 months. It is the first reported case of cervical MPNST receiving combined treatment.