Primary EWS/PNET of the lung with TP53 germline and SKT11 somatic mutation: A case report and review of the literature

Primary pulmonary EWS/PNET is extremely rare. This report describes a 20 year‐old man with primary pulmonary EWS/PNET with TP53 germline and SKT11 somatic mutation. After four neoadjuvant chemotherapy cycles (VAC with alternating IE) combined with anlotinib, a left pneumonectomy was performed. Maint...

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Detalles Bibliográficos
Autores principales: Zhang, Shuangping, Chen, Yun, Guo, Shiping, Chen, Ke‐Neng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons Australia, Ltd 2021
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8720619/
https://www.ncbi.nlm.nih.gov/pubmed/34866350
http://dx.doi.org/10.1111/1759-7714.14251
Descripción
Sumario:Primary pulmonary EWS/PNET is extremely rare. This report describes a 20 year‐old man with primary pulmonary EWS/PNET with TP53 germline and SKT11 somatic mutation. After four neoadjuvant chemotherapy cycles (VAC with alternating IE) combined with anlotinib, a left pneumonectomy was performed. Maintenance anlotinib monotherapy was then continued with no sign of recurrence to date. It is suggested that before the treatment and prognosis of children or young adults with primary EWS/PNET of the lung that consideration should be given to genetic testing.

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