Primary pulmonary epithelioid malignant peripheral nerve sheath tumor in a child: a case report

Moyan Zhang; Peng Song; Lin Yang; Wei Guo; Ying Ji; Huayu He; Shugeng Gao

doi:10.21037/atm.2020.02.56

Primary pulmonary epithelioid malignant peripheral nerve sheath tumor in a child: a case report

Ann Transl Med. 2020 Mar;8(6):403. doi: 10.21037/atm.2020.02.56.

Authors

Moyan Zhang¹, Peng Song¹, Lin Yang², Wei Guo¹, Ying Ji¹, Huayu He¹, Shugeng Gao¹

Affiliations

¹ 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.
² 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.

Abstract

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.

Keywords: Malignant peripheral nerve sheath tumor (MPNST); child; lung; surgery.

Publication types

Case Reports