Open AccessPrimary EWS / PNET of the lung with TP53 germline and SKT11 somatic mutation: A case report and review of the literature
Author(s) -
Zhang Shuangping,
Chen Yun,
Guo Shiping,
Chen KeNeng
Publication year - 2022
Publication title -
thoracic cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.823
H-Index - 28
eISSN - 1759-7714
pISSN - 1759-7706
DOI - 10.1111/1759-7714.14251
Subject(s) - medicine , germline , mutation , somatic cell , germline mutation , lung , genetics , gene , biology
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.