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Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

BACKGROUND: Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely ra...

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Autores principales: Xu, Qiong-Qian, Xing, Wen-Wen, Chen, Gang, Dang, Yi-Wu, Luo, Yi-Ge, Chen, Peng, Liang, Song-Wu, Chen, Jia-Bo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854414/
https://www.ncbi.nlm.nih.gov/pubmed/31750352
http://dx.doi.org/10.12998/wjcc.v7.i21.3671
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author Xu, Qiong-Qian
Xing, Wen-Wen
Chen, Gang
Dang, Yi-Wu
Luo, Yi-Ge
Chen, Peng
Liang, Song-Wu
Chen, Jia-Bo
author_facet Xu, Qiong-Qian
Xing, Wen-Wen
Chen, Gang
Dang, Yi-Wu
Luo, Yi-Ge
Chen, Peng
Liang, Song-Wu
Chen, Jia-Bo
author_sort Xu, Qiong-Qian
collection PubMed
description BACKGROUND: Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva. CASE SUMMARY: Case 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm. The patient underwent radical resection of the tumor. After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide (IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression. Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm. The tumor was partially resected by surgery. The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy, and the patient died at home six months after surgery. CONCLUSION: PNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis.
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spelling pubmed-68544142019-11-20 Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature Xu, Qiong-Qian Xing, Wen-Wen Chen, Gang Dang, Yi-Wu Luo, Yi-Ge Chen, Peng Liang, Song-Wu Chen, Jia-Bo World J Clin Cases Case Report BACKGROUND: Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva. CASE SUMMARY: Case 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm. The patient underwent radical resection of the tumor. After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide (IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression. Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm. The tumor was partially resected by surgery. The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy, and the patient died at home six months after surgery. CONCLUSION: PNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis. Baishideng Publishing Group Inc 2019-11-06 2019-11-06 /pmc/articles/PMC6854414/ /pubmed/31750352 http://dx.doi.org/10.12998/wjcc.v7.i21.3671 Text en ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.
spellingShingle Case Report
Xu, Qiong-Qian
Xing, Wen-Wen
Chen, Gang
Dang, Yi-Wu
Luo, Yi-Ge
Chen, Peng
Liang, Song-Wu
Chen, Jia-Bo
Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title_full Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title_fullStr Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title_full_unstemmed Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title_short Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
title_sort primitive neuroectodermal tumors of the abdominal wall and vulva in children: report of two cases and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854414/
https://www.ncbi.nlm.nih.gov/pubmed/31750352
http://dx.doi.org/10.12998/wjcc.v7.i21.3671
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