PDF(Pubmed)
Abstract：
Primary pulmonary malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma with a low incidence, poor prognosis and limited treatment options. The present study reported a case of lung MPNST in a 63-year-old male patient without any pulmonary symptoms. Immunohistochemical analysis of the tumor indicated a programmed death-ligand 1 (PD-L1) expression tumor proportion score of 60%. A total of six courses of sintilimab were used in this patient and a remarkable response was achieved. In summary, sintilimab single-agent immunotherapy may be a novel treatment for pulmonary MPNST. When encountering analogous cases in the future, oncologists can test for the expression of PD-L1 in patients to guide the therapy\'s design.
摘要：
原发性肺恶性外周神经鞘瘤(MPNST)是一种少见的软组织肉瘤,发病率低,预后差，治疗选择有限。本研究报告了一名63岁男性患者的肺部MPNST病例，没有任何肺部症状。肿瘤的免疫组织化学分析表明程序性死亡配体1(PD-L1)表达肿瘤比例评分为60%。该患者总共使用了六个疗程的sintilimab，并取得了显着的反应。总之,sintilimab单药免疫治疗可能是一种新的治疗肺部MPNST的方法。将来遇到类似案例时，肿瘤学家可以检测患者PD-L1的表达，以指导治疗的设计。
