Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 6, 2019; 7(3): 340-346
Published online Feb 6, 2019. doi: 10.12998/wjcc.v7.i3.340
Long-term survival of a patient with a large adrenal primitive neuroectodermal tumor: A case report
Jun Dai, Hong-Chao He, Xin Huang, Fu-Kang Sun, Yu Zhu, Dan-Feng Xu
Jun Dai, Hong-Chao He, Xin Huang, Fu-Kang Sun, Yu Zhu, Dan-Feng Xu, Department of Urology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200025, China
Author contributions: Dai J, He HC, and Sun FK conceived the study; Dai J and He HC searched the published articles, analyzed the data, and wrote the manuscript; Huang X, Zhu Y, and Xu DF collected the case history and data; Sun FK reviewed and confirmed the final version of the manuscript and provided funding.
Supported by National Natural Science Foundation of China, No. 81572621; and Medical and Technology Intercrossing Research Foundation of Shanghai Jiaotong University, No. YG2016QN65.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Fu-Kang Sun, MD, Professor, Department of Urology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, No. 197, Ruijin Er Road, Shanghai 200025, China. sunfukang6@126.com
Telephone: +86-21-64370045-666064 Fax: +86-21-64315370
Received: October 12, 2018
Peer-review started: October 13, 2018
First decision: October 18, 2018
Revised: November 11, 2018
Accepted: November 23, 2018
Article in press: November 24, 2018
Published online: February 6, 2019
Abstract
BACKGROUND

Adrenal primitive neuroectodermal tumor (PNET) is an extremely rare malignant tumor with poor prognosis and of neural crest origin. Herein, we report a case of adrenal PNET and summarized its clinical and pathological characteristics on the basis of 16 patients reported recently.

CASE SUMMARY

A female patient aged 25 years presented with right lumbago for 12 mo, and pre-operative computed tomography showed a huge right adrenal mass. She received tumorectomy, and post-operative pathological examination showed adrenal PNET. After surgery, she underwent adjuvant chemotherapy and was followed up 31 mo after surgery. She received brachytherapy for right paracolic and hepatic metastases. She was alive and followed up for 60 mo. In available studies, only 57.14% (4/7) and 44.44% (4/9) were positive for the expression of neuron-specific enolase and synaptophysin, respectively, although CD99 expression was found in all the patients (100%; 10/10).

CONCLUSION

It is concluded that adrenal PNET is very rare and highly malignant, and histology is a golden standard in its diagnosis. Surgery and adjuvant therapy is the main treatment.

Keywords: Primitive neuroectodermal tumor, Adrenal gland, Case report

Core tip: Primitive neuroectodermal tumor (PNET), a member of small round blue cell tumors, is very rare and highly malignant. PNET originating from the adrenal is extremely rare and only a few cases have been reported. The outcome of adrenal PNET is poor, standard treatments have never been established, and the efficacy of adjuvant chemotherapy and radiotherapy after operation is still unclear. Herein, we reported a case of adrenal PNET in a female patient who was alive after 60 mo of follow-up. We hypothesize that early removal and chemotherapy provided the excellent outcome of the patient.