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Original Article
Clinical Significance of Random Urinary Vanillylmandelic Acid in Patients with Neuroblastoma
Clin Pediatr Hematol Oncol 2018;25:142-8.
Published online October 31, 2018
© 2018 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Esther Park, M.D.1, Hyojung Park, M.D.1, Heewon Cho, M.D.1, Youngeun Ma, M.D.1, Soo-Youn Lee, M.D.2, Ji Won Lee, M.D.1, Keon Hee Yoo, M.D.1, Ki Woong Sung, M.D.1 and Hong Hoe Koo, M.D.1

Departments of 1Pediatrics and 2Laboratory Medicine and Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Correspondence to: Youngeun Ma
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea
Tel: +82-2-3410-3537
Fax: +82-2-3410-0043
E-mail: jeajgs@gmail.com
ORCID ID: orcid.org/0000-0002-5862-6319
Received July 2, 2018; Revised July 24, 2018; Accepted September 3, 2018.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Background: To evaluate the value of random urinary vanillylmandelic acid (VMA) as a surrogate marker for monitoring tumor response and predicting outcome in patients with neuroblastoma (NB).
Methods: Medical records of 91 patients newly diagnosed with NB at the Samsung Medical Center between June 2014 and August 2017 were reviewed. Clinical associations and other prognostic factors, including age at diagnosis, stage, pathologic subtype, MYCN amplification, and other cytogenetic aberrations, were analyzed. Furthermore, the significance of random urinary VMA level in predicting outcome and tumor response was also evaluated.
Results: The median random urinary VMA level at diagnosis was 27.9 (range: 1.7-600)mg/g creatinine. Abdominal primary site, male sex, advanced stage, less differentiated pathology (poorly differentiated, undifferentiated), 11q deletion, and high-risk tumor were associated with a higher VMA level at diagnosis. The VMA level decreased during chemotherapy (28.4%, 16.9%, and 9.6% of the VMA level at diagnosis after 3, 6, and 9 cycles of chemotherapy, respectively). A higher VMA level at diagnosis tends to be associated with a better overall survival in high-risk patients with borderline significance (58.3±18.6% vs. 76.5±13.4%, P=0.050). However, in the multivariate analysis, the VMA level was not a significant predictor of survival. A slower reduction in VMA level during chemotherapy was not associated with a worse overall survival. However, event free survival was significantly better in the rapid responder group.
Conclusion: A higher VMA level was associated with high-risk features at diagnosis of NB. Random urinary VMA is a valuable marker for monitoring NB response during chemotherapy.
Keywords: Neuroblastoma, Vanillylmandelic acid, Prognosis, Survival


October 2018, 25 (2)
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  • Youngeun Ma