Department of Medical Oncology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui, China.
J Coll Physicians Surg Pak. 2022 Apr;32(4):S24-S27. doi: 10.29271/jcpsp.2022.Supp1.S24.
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. Key Words: Malignant peripheral nerve sheath tumor, Cervix, Pulmonary metastasis, Hysterectomy, Chemotherapy.
女性宫颈原发性恶性外周神经鞘瘤且不伴 1 型神经纤维瘤病极为罕见。本文报告了 1 例 46 岁女性宫颈恶性外周神经鞘瘤（MPNST）病例。患者因不规则阴道出血就诊，盆腔 CT 提示宫颈占位，行根治性子宫切除术。术后组织病理学和免疫组化检查诊断为 MPNST。1 年后，患者复发并发生肺转移，接受表柔比星/异环磷酰胺、达卡巴嗪、依托泊苷/顺铂多线化疗。MPNST 预后差，与侵袭性、高复发和转移率有关。该患者接受了手术、多线化疗和靶向治疗，但总生存期（OS）为 44 个月。这是首例报道宫颈 MPNST 接受联合治疗的病例。关键词：恶性外周神经鞘瘤、宫颈、肺转移、子宫切除术、化疗。
