Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2020; 8(9): 1685-1692
Published online May 6, 2020. doi: 10.12998/wjcc.v8.i9.1685
Central nervous system relapse in a pediatric anaplastic large cell lymphoma patient with CLTC/ALK translocation treated with alectinib: A case report
Jing Yang, Jun Li, Wei-Yue Gu, Ling Jin, Yan-Long Duan, Shuang Huang, Meng Zhang, Xi-Si Wang, Yi Liu, Chun-Ju Zhou, Chao Gao, Hu-Yong Zheng, Yong-Hong Zhang
Jing Yang, Jun Li, Ling Jin, Yan-Long Duan, Shuang Huang, Meng Zhang, Xi-Si Wang, Yi Liu, Chun-Ju Zhou, Chao Gao, Hu-Yong Zheng, Yong-Hong Zhang, Beijing Key Laboratory of Pediatric Hematology Oncology, National Discipline of Pediatrics, Ministry of Education, MOE Key Laboratory of Major Diseases in Children; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China
Wei-Yue Gu, Chigene (Beijing) Translational Medical Research Center Co., Ltd., Beijing 101111, China
Author contributions: Zheng HY, Zhang YH, and Yang J designed the study; Li J, Gu WY, Liu Y, Zhou CJ, and Gao C performed the experiments; Yang J, Li J, Duan YL, Huang S, Zhang M, and Wang XS collected the data and samples; Yang J, Li J, and Gao C wrote the manuscript; Zheng HY and Zhang YH reviewed the final manuscript and take primary responsibility for the article; all authors read and approved the final manuscript.
Supported by the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority, No. XTZD20180204.
Informed consent statement: Informed written consent was obtained from the patient and her parents for publication of this report and the accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict 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 that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: Hu-Yong Zheng, MD, PhD, Beijing Key Laboratory of Pediatric Hematology Oncology, National Discipline of Pediatrics, Ministry of Education, MOE Key Laboratory of Major Diseases in Children; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children’s Health, No. 56,Nanlishi Road, Beijing 100045, China. zhenghuyong@vip.sina.com
Received: January 16, 2020
Peer-review started: January 16, 2020
First decision: March 27, 2020
Revised: April 8, 2020
Accepted: April 21, 2020
Article in press: April 21, 2020
Published online: May 6, 2020
Processing time: 105 Days and 3.5 Hours
Abstract
BACKGROUND

The aberrant expression of the anaplastic lymphoma kinase (ALK) gene in ALK-positive (ALK+) anaplastic large cell lymphoma (ALCL) is usually due to t(2;5)/NPM-ALK. However, rarely, aberrant ALK expression can also result from a rearrangement of the ALK gene with various partner genes. Central nervous system (CNS) metastasis is very rare in ALK+ALCL. Patients with CNS involvement show an inferior prognosis.

CASE SUMMARY

Here, we present the case of an 8-year-old girl diagnosed with ALK+ALCL. She presented with fever, skin nodules, leg swelling, and abdominal pain over the preceding 6 mo. She had extensive involvement and showed an extraordinary rare translocation, t(2;17)/CLTC-ALK, as demonstrated by RNA-seq. She underwent chemotherapy as per ALCL99, followed by vinblastine (VBL) maintenance treatment, and achieved complete remission. However, she developed CNS relapse during VBL monotherapy. The patient achieved a durable second remission with high-dose chemotherapy (including methotrexate 8 g/m2) and continuous treatment with alectinib and VBL.

CONCLUSION

Alectinib showed significant and durable CNS effects in this patient. However, more cases are needed to prove the efficacy and safety of alectinib for pediatric ALK+ALCL patients.

Keywords: Anaplastic; Lymphoma; Pediatric; CLTC/ALK; Central nervous system; Alectinib; Case report

Core tip: Both CLTC-anaplastic lymphoma kinase translocation and central nervous system (CNS) metastasis are very rare in anaplastic large cell lymphoma. This paper reports a rare pediatric case of anaplastic large cell lymphoma with the CLTC-anaplastic lymphoma kinase fusion gene. The patient had an aggressive clinical course and underwent CNS relapse during treatment. The current patient achieved sustained complete remission with chemotherapy and alectinib. Alectinib conferred significant and durable CNS effects in this patient.