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

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

doi:10.3892/ol.2024.14556

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

Authors:
- Yunqi Chen
- Ting Chen
- Wanshan Zhu
- Luzhen Li
- Cantu Fang
- Huatang Zhang
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Affiliations: Oncology Department, Zhongshan Hospital of Traditional Chinese Medicine Affiliated to Guangzhou University of Traditional Chinese Medicine, Zhongshan, Guangdong 528400, P.R. China
Published online on: July 4, 2024 https://doi.org/10.3892/ol.2024.14556
Article Number: 423
Copyright: © Chen et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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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.

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