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罕见的原发性肺内恶性周围神经鞘瘤对信迪利单抗显示出显著反应:一例报告及文献综述

Rare primary intrapulmonary malignant peripheral nerve sheath tumor showing significant response to sintilimab: A case report and literature review.

作者信息

Chen Yunqi, Chen Ting, Zhu Wanshan, Li Luzhen, Fang Cantu, Zhang Huatang

机构信息

Oncology Department, Zhongshan Hospital of Traditional Chinese Medicine Affiliated to Guangzhou University of Traditional Chinese Medicine, Zhongshan, Guangdong 528400, P.R. China.

出版信息

Oncol Lett. 2024 Jul 4;28(3):423. doi: 10.3892/ol.2024.14556. eCollection 2024 Sep.

DOI:10.3892/ol.2024.14556
PMID:39035047
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11258603/
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

原发性肺恶性周围神经鞘瘤(MPNST)是一种罕见的软组织肉瘤,发病率低,预后差,治疗选择有限。本研究报告了一例63岁男性肺MPNST患者,该患者无任何肺部症状。对肿瘤进行免疫组织化学分析显示,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信迪利单抗,取得了显著疗效。总之,信迪利单抗单药免疫治疗可能是肺MPNST的一种新的治疗方法。未来遇到类似病例时,肿瘤学家可以检测患者的PD-L1表达,以指导治疗方案的设计。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/dffe8df48d8c/ol-28-03-14556-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/f18e0e8f420f/ol-28-03-14556-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/2389be69bf5f/ol-28-03-14556-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/dffe8df48d8c/ol-28-03-14556-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/f18e0e8f420f/ol-28-03-14556-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/2389be69bf5f/ol-28-03-14556-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c0b/11258603/dffe8df48d8c/ol-28-03-14556-g02.jpg

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Deep Response to Anti-PD-1 Therapy of Metastatic Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumor With Amplification.
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