Background: Opsoclonus-myoclonus syndrome (OMS) is an immune-mediated disorder that affects the central nervous system. It is recognized as a paraneoplastic syndrome highly associated with neuroblastic tumors. In Mexico, accurate epidemiological data are lacking and there are no well-structured protocols aimed at standardizing a treatment regimen in patients with OMS. Aim: This study aims to describe the clinical features, etiological factors, and treatment related to OMS in Mexican children. Patients and Methods: A retrospective study was performed over a 10-year period (2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016), using the database of the "Hospital Infantil de México Federico Gómez". The patients were divided into two groups (tumor vs. no tumor) to determine if there were significant demographic differences between the subgroups. The demographic variables were compared between groups using the Student's t-test. Results: Eleven patients were identified. The median age at the onset of the first clinical manifestation was 23 ± 14 months and the median age at diagnosis was 26 ± 15 months. The time delay between the onset of symptoms and diagnosis was approximately 3 months. Conclusion: In Mexico, there is a paucity of experience regarding the clinical presentation of OMS, and due to the infrequency of this condition, it is rarely diagnosed in our population since most pediatricians are unaware of the existence of the syndrome. The findings of this paper are consistent with the previous studies in terms of a closely equal sex ratio, predominance of Stage I tumors, a greater number of female patients with a paraneoplastic etiology, high relapse rates, and frequent neuropsychiatric morbidity.
Introduction: Due to progress in immunohistochemistry and the growing understanding of gastrointestinal stromal tumors (GISTs), it has been possible to elucidate certain aspects related to this group of neoplasms, but, given its unusual characteristics, new questions arose. Such is the case of the subtype called "wild-type GIST" (WT-GIST) (GIST, which lacks the mutations (KIT or PDGFRA). They represent a therapeutic challenge due to their resistance to agents that act as a molecular target for the disease. The aim of this study was to make a brief analysis of the WT-GIST. Material and methods: A bibliographic review of the PubMed and ResearchGate database was conducted. The MeSH terms "GIST" AND "Wild-type" OR "pediatric" were used. Studies published between 2005 and 2018 were cataloged, and originals, reviews, and editorials in English were selected. Given that the aim of the study was bibliographical, there was no need to perform a formal statistical analysis. Results: When comparing WT-GISTs with the classic phenotype, wild tumors have a set of non-specific heterogeneous mutations such as BRAF, NF-1, KRAS, CBL, PI3K3CA, or protein succinate dehydrogenase (subunits A, B, C, and D), this last one being the most common. Conclusions: Nowadays, given the unique and indolent characteristics of this group of neoplasms, there is a need for the correct diagnosis and limitation of the subgroup to which they belong, to reduce the risk of failure for the various existing therapeutic pathways.
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