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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Baishideng Publishing Group Inc
2019
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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. |
format | Online Article Text |
id | pubmed-6854414 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
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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