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Anand S, Agarwala S, Jain V, et al. Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting. J Pediatr Hematol Oncol. 2021;43(7):e924-e929doi: 10.1097/MPH.0000000000002131.
Anand, S., Agarwala, S., Jain, V., Bakhshi, S., Dhua, A., Gulati, S., Seth, R., Srinivas, M., Jana, M., Kandasamy, D., & Bhatnagar, V. (2021). Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting. Journal of pediatric hematology/oncology, 43(7), e924-e929. https://doi.org/10.1097/MPH.0000000000002131
Anand, Sachit, et al. "Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting." Journal of pediatric hematology/oncology vol. 43,7 (2021): e924-e929. doi: https://doi.org/10.1097/MPH.0000000000002131
Anand S, Agarwala S, Jain V, Bakhshi S, Dhua A, Gulati S, Seth R, Srinivas M, Jana M, Kandasamy D, Bhatnagar V. Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting. J Pediatr Hematol Oncol. 2021 Oct 01;43(7):e924-e929. doi: 10.1097/MPH.0000000000002131. PMID: 33769388.
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J Pediatr Hematol Oncol. 2021 Oct 01;43(7):e924-e929. doi: 10.1097/MPH.0000000000002131.

Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting.

Journal of pediatric hematology/oncology

Sachit Anand, Sandeep Agarwala, Vishesh Jain, Sameer Bakhshi, Anjan Dhua, Sheffali Gulati, Rachna Seth, Maddur Srinivas, Manisha Jana, Devasenathipathy Kandasamy, Veereshwar Bhatnagar

Affiliations

  1. Departments of Pediatric Surgery.
  2. Department of Medical Oncology, BRAIRCH, AIIMS, New Delhi, India.
  3. Pediatrics.
  4. Radiodiagnosis.

PMID: 33769388 DOI: 10.1097/MPH.0000000000002131

Abstract

Children with neuroblastoma (NB) and opsoclonus-myoclonus-ataxia syndrome (OMAS) have a favorable oncologic outcome and overall survival. In contrast, despite intensive multidrug immunomodulation, the neurologic outcome is complicated by the relapsing nature of the neurologic symptoms and long-term neurobehavioral sequelae. Being associated with low-risk NB, there exists an ambiguity in the current literature regarding the administration of chemotherapy in these children. We reviewed our archives for children with NB-OMAS over a 22-year (January 1996 to January 2018) period. Eighteen children (10 female) with a median age at diagnosis of 23 months had NB-OMAS and were included. They had stage 1 (9/18; 50%), 2 (1/18; 5.5%), 3 (7/18; 39%), and 4 (1/18; 5.5%) disease according to the International Neuroblastoma Staging System. Multimodality therapy included surgery (16/18; 89%), chemotherapy (11/18; 61%), and immunomodulatory therapy (10/18; 55%). Complete oncologic remission was achieved in all children. Relapse of OMAS and presence of neurologic sequelae were observed in 1 (5.5%) and 5 (28%) cases, respectively. Presence of neurologic sequelae was significantly associated with low-tumor stage (P=0.036) and treatment without chemotherapy (P=0.003). Chemotherapy administration was the only variable significantly predicting a favorable neurologic outcome (95% confidence interval: 0.26-1.40, P=0.01). To conclude, our study including a limited cohort of patients highlights a favorable neurologic outcome associated with chemotherapy administration in children with NB-OMAS. However, further studies with larger sample size need to be conducted before drawing any definite conclusions.

Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

Conflict of interest statement

The authors declare no conflict of interest.

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