title: Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review.
abstract: Primitive neuroectodermal tumors (PNETs) arising directly from the lung are extremely rare but particularly aggressive neoplasms. Although thoracic PNET usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 16-year-old male with PNET diagnosed following histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy. As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy. We attempted to explore the use of targeted pharmacotherapy through high-throughput sequencing in this case. We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.
authors.affiliation: From the Department of Lung Cancer Surgery, Tianjin Lung Cancer Institute (MD, JL, ZS, XL, DR, JC); Department of Pathology; Tianjin Medical University General Hospital, Heping District, Tianjin, China (TS, DW).
authors.forename: Ming
authors.initials: M
authors.lastname: Dong
authors.forename: Jinghao
authors.initials: J
authors.lastname: Liu
authors.forename: Zuoqing
authors.initials: Z
authors.lastname: Song
authors.forename: Xin
authors.initials: X
authors.lastname: Li
authors.forename: Tao
authors.initials: T
authors.lastname: Shi
authors.forename: Dan
authors.initials: D
authors.lastname: Wang
authors.forename: Dian
authors.initials: D
authors.lastname: Ren
authors.forename: Jun
authors.initials: J
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000235:genetics; D008297:Male; D009154:Mutation; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000235:genetics; D011379:Prognosis; D014057:Tomography, X-Ray Computed