Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 6, 2019; 7(21): 3671-3682
Published online Nov 6, 2019. doi: 10.12998/wjcc.v7.i21.3671
Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature
Qiong-Qian Xu, Wen-Wen Xing, Gang Chen, Yi-Wu Dang, Yi-Ge Luo, Peng Chen, Song-Wu Liang, Jia-Bo Chen
Qiong-Qian Xu, Yi-Ge Luo, Peng Chen, Song-Wu Liang, Jia-Bo Chen, Department of Pediatric Surgery, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
Wen-Wen Xing, Gang Chen, Yi-Wu Dang, Department of Pathology, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
Author contributions: Chen G, Dang YW, Luo YG, and Chen JB designed the research, supervised all experiments and corrected the manuscript; Xu QQ, Xing WW , Chen P, and Liang SW collected the clinical information, summarized literature, and performed histologic examination and immunohistochemistry; and Xu QQ wrote first version of the paper.
Supported by the Guangxi Natural Science Foundation Project, No. 2014GXNSFAA118202.
Informed consent statement: The patients provided informed consent for publication of the case.
Conflict-of-interest statement: The authors of this manuscript have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The manuscript was revised according to the CARE checklist.
Open-Access: 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. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Jia-Bo Chen, MD, Doctor, Department of Pediatric Surgery, the First Affiliated Hospital of Guangxi Medical University, No. 6, Shuangyong Road, Nanning 530021, Guangxi Zhuang Autonomous Region, China. cjb1205@163.com
Telephone: +86-0771-5337362
Received: June 29, 2019
Peer-review started: July 1, 2019
First decision: July 31, 2019
Revised: August 25, 2019
Accepted: September 13, 2019
Article in press: September 12, 2019
Published online: November 6, 2019
Processing time: 132 Days and 22 Hours
Abstract
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.

Keywords: Primitive neuroectodermal tumor; Therapy; Prognosis; Case report

Core tip: Primitive neuroectodermal tumors (PNETs) are rare undifferentiated tumors with similar biological characteristics. They belong to the Ewing sarcoma family, accounting for 4% to 17% of all pediatric soft tissue tumors. PNETs usually occur in children and young adults under 25 years of age. We retrospectively analyzed two PNET cases at the First Affiliated Hospital of Guangxi Medical University from May 2012 to June 2014. Both patients were female with an age of onset at 66 and 40 mo. Both patients were provided inpatient visits, outpatient medical records, and telephone follow-ups for more than one year. In this report, we describe in detail the clinical manifestations, treatment protocols, pathological findings, and patient prognoses. This report provides an in-depth analysis of two cases of PNET at rare sites.