PDF(Pubmed)
Abstract：
Malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue sarcoma with a poor prognosis, affecting most commonly the extremities. The lungs constitute the most frequent location for distant metastases. Half of all MPNSTs arise in patients with neurofibromatosis type 1, while approximately 10% are radiation induced and the rest are sporadic.The authors present a pregnant woman in her 40s with a sporadic MPNST of the lower limb and with lung metastases at diagnosis. Treatment consisted of interilioabdominal amputation, followed by adjuvant chemotherapy. Partial response and disease stabilisation were achieved with chemotherapy.Surgical resection with negative margins is the only potentially curative therapy, while radiation therapy and chemotherapy might be useful in the neoadjuvant or adjuvant setting, but their advantage in survival is not demonstrated. In the reported case, chemotherapy permitted the achievement of partial response and stabilisation of the disease.
摘要：
恶性外周神经鞘瘤（MPNST）是一种侵袭性软组织肉瘤，预后不良，最常见的影响四肢。肺构成远处转移的最常见位置。所有MPNST的一半出现在1型神经纤维瘤病患者中，而大约10％是辐射诱导的，其余的是散发性的。作者介绍了一名40多岁的孕妇，下肢有零星的MPNST，诊断时有肺转移。治疗包括髂内腹部截肢，其次是辅助化疗。化疗可实现部分反应和疾病稳定。切缘阴性的手术切除是唯一可能治愈的治疗方法，虽然放疗和化疗可能在新辅助或辅助治疗中有用，但是他们在生存方面的优势没有得到证明。在报告的案例中，化疗允许实现部分反应和疾病的稳定。
