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

• Published in: Thoracic cancer Vol. 13; no. 1; pp. 137 - 140
• Main Authors: Zhang, Shuangping, Chen, Yun, Guo, Shiping, Chen, Ke‐Neng
• Format: Journal Article
• Language: English
• Published: Melbourne John Wiley & Sons Australia, Ltd 01-01-2022; John Wiley & Sons, Inc; Wiley
• Subjects: Case Report; Case Reports; Chemotherapy; Combined Modality Therapy; Conflicts of interest; Ewing's sarcoma; Ewings sarcoma; Germ-Line Mutation; Humans; Lymphatic Metastasis - genetics; Lymphatic Metastasis - therapy; Lymphatic system; Male; Medical prognosis; Metastasis; Microscopy; Mutation; Neuroectodermal Tumors, Primitive - genetics; Neuroectodermal Tumors, Primitive - therapy; Pneumonectomy; Pneumonectomy - methods; Proteins - genetics; pulmonary; Pulmonary arteries; Radiation therapy; RNA-Binding Protein EWS - genetics; Sarcoma, Ewing - genetics; Sarcoma, Ewing - therapy; Surgery; TP53; Tumor Suppressor Protein p53 - genetics; Tumors; Veins & arteries; Whole Exome Sequencing; Young Adult; pulmonary; TP53; Ewing's sarcoma
• ISSN: 1759-7706; 1759-7714
• DOI: 10.1111/1759-7714.14251

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.
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 4 neoadjuvant therapy, R0 resection was obtained. Then maintenance anlotinib monotherapy was continued, no sign of recurrence to date as an outcome.