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

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 patie...

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Detalles Bibliográficos
Autores principales: Zhang, Moyan, Song, Peng, Yang, Lin, Guo, Wei, Ji, Ying, He, Huayu, Gao, Shugeng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: AME Publishing Company 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186698/
https://www.ncbi.nlm.nih.gov/pubmed/32355847
http://dx.doi.org/10.21037/atm.2020.02.56
Descripción
Sumario: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.

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