Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review

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 wi...

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Autores principales: Dong, Ming, Liu, Jinghao, Song, Zuoqing, Li, Xin, Shi, Tao, Wang, Dan, Ren, Dian, Chen, Jun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4504587/
https://www.ncbi.nlm.nih.gov/pubmed/26166119
http://dx.doi.org/10.1097/MD.0000000000001136
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author Dong, Ming
Liu, Jinghao
Song, Zuoqing
Li, Xin
Shi, Tao
Wang, Dan
Ren, Dian
Chen, Jun
author_facet Dong, Ming
Liu, Jinghao
Song, Zuoqing
Li, Xin
Shi, Tao
Wang, Dan
Ren, Dian
Chen, Jun
author_sort Dong, Ming
collection PubMed
description 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.
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spelling pubmed-45045872015-08-05 Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review Dong, Ming Liu, Jinghao Song, Zuoqing Li, Xin Shi, Tao Wang, Dan Ren, Dian Chen, Jun Medicine (Baltimore) 6700 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. Wolters Kluwer Health 2015-07-13 /pmc/articles/PMC4504587/ /pubmed/26166119 http://dx.doi.org/10.1097/MD.0000000000001136 Text en Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 6700
Dong, Ming
Liu, Jinghao
Song, Zuoqing
Li, Xin
Shi, Tao
Wang, Dan
Ren, Dian
Chen, Jun
Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title_full Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title_fullStr Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title_full_unstemmed Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title_short Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
title_sort primary multiple pulmonary primitive neuroectodermal tumor: case report and literature review
topic 6700
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4504587/
https://www.ncbi.nlm.nih.gov/pubmed/26166119
http://dx.doi.org/10.1097/MD.0000000000001136
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