Zhang Moyan, Song Peng, Yang Lin, Guo Wei, Ji Ying, He Huayu, Gao Shugeng
Department of Thoracic Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Ann Transl Med. 2020 Mar;8(6):403. doi: 10.21037/atm.2020.02.56.
Primary pulmonary malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas originating from intrapulmonary nerve sheath and have been rarely observed in children. Here, we report a case of a 16-year-old child who presented with a mass located in the upper lobe of right lung. The patient underwent a sleeve lobectomy and the pathological diagnosis of the tumor was primary pulmonary epithelioid MPNST. Despite the radical resection, multiple suspected metastasis occurred in heart, lung and muscles less than 2 years after the operation and the patient died 6 months after the metastasis.
原发性肺恶性周围神经鞘瘤（MPNSTs）是起源于肺内神经鞘的罕见肉瘤，在儿童中很少见。在此，我们报告一例16岁儿童，其右肺上叶出现肿块。患者接受了袖状肺叶切除术，肿瘤的病理诊断为原发性肺上皮样MPNST。尽管进行了根治性切除，但术后不到2年，心脏、肺和肌肉出现多处疑似转移，患者在转移后6个月死亡。
