Clinical features and long-term sequelae among children with neuroblastoma and opsoclonus-myoclonus syndrome at diagnosis: Experience of the Instituto Nacional de Pediatria, Mexico City, Mexico.

Castellanos-Toledo, Araceli; Portillo-Zavala, Claudia Selene; Cárdenas-Cardós, Rocío; Carbajal, Jose de Jesus Figueroa

doi:10.1200/jco.2018.36.15_suppl.e22509

Cited by 2 publications

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“…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. Similar to the findings reported by Castellanos-Toledo et al 16 (23.1 months, range: 11-47 months), we observed a higher median age at clinical presentation (22.6 months, range: 9-51 months) in Mexican children. Moreover, the average duration of therapy was nearly equal in both studies (33 vs. 32.36 ± 26.95 months), but there was a lower median time in symptom duration (4.3 months vs. 2.45 ± 1.9 months).…”

Section: Discussionsupporting

confidence: 91%

“…Moreover, the average duration of therapy was nearly equal in both studies (33 vs. 32.36 ± 26.95 months), but there was a lower median time in symptom duration (4.3 months vs. 2.45 ± 1.9 months). When comparing clinical features between the etiological subgroups in our study with the information reported by Castellano-Toledo et al 16 , we found that they reported no gender differences in the incidence of NB, while our study showed a clear predominance of females in the tumor group. Furthermore, the primary localization of NB was the adrenal gland (five cases), while there was only one case localized in the paravertebral area (mediastinum).…”

Section: Discussionsupporting

confidence: 71%

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Clinical features of 11 patients with opsoclonus-myoclonus syndrome diagnosed in an urban pediatric institution in Mexico City: A retrospective study

Castañón-González¹,

Barragán-Pérez²,

González-Negrete³

et al. 2019

RMU

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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.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 71%

Clinical features of 11 patients with opsoclonus-myoclonus syndrome diagnosed in an urban pediatric institution in Mexico City: A retrospective study

Castañón-González¹,

Barragán-Pérez²,

González-Negrete³

et al. 2019

RMU

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“…The incidence of NBL associated with the rare paraneoplastic OMAS is ∼3%. 5,16 In the present study, data of a single-center "CCHE" was presented for 15 eligible OMAS patients with a frequency around 1% of all NBL cases that was exclusively of LR to IR and favorable survival outcome. All patients remained alive with a 100 ± 0% 5-year OS and 85.7 ± 9.4 EFS.…”

Section: Discussionmentioning

confidence: 99%

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

Elzomor

Menawi

ElAwady

et al. 2022

Journal of Pediatric Hematology/Oncology

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Opsoclonous myoclonous ataxia syndrome (OMAS) is a rare primarily immune-mediated disease in children. The current study aim was to find out the patterns and outcome of OMAS associated with neuroblastoma (NBL) among Children’s Cancer Hospital—Egypt patients. Data was reviewed for 15 eligible patients enrolled between 2007 and 2016. OMAS treatment included prednisolone and cyclophosphamide with/without intravenous immunoglobulin; NBL treatment was given according to risk-corresponding protocol. Patients’ age ranged from 0.75 to 12 years at presentation with male/female: 1.1/1. Concurrent diagnosis of OMAS and NBL occurred in 6 patients (40%). OMAS preceded NBL within 0.25 to 2 years in 33%, while NBL preceded OMAS within 0.5 to 1.5 years in 27%. Full OMAS picture was present in 10/15 patients, while 20% presented with truncal ataxia and myoclonus, 1 with truncal ataxia and opsoclonus, and 1 had opsoclonus and myoclonus. Median time till improvement of manifestations was 5 months. The 5-year OMAS progression-free survival was 33.3%, where 10 patients needed second-line therapy due to relapse/progression of OMAS. The median time to progression was 28 months measured from OMAS diagnosis. All patients remained alive with NBL 5-year overall survival of 100% and event-free survival of 85.7% for. However, 73% of the patients showed late sequelae ranging from ocular to cognitive, behavioral and motor disorders; rarely seizures and hemolytic anemia.

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Clinical features and long-term sequelae among children with neuroblastoma and opsoclonus-myoclonus syndrome at diagnosis: Experience of the Instituto Nacional de Pediatria, Mexico City, Mexico.

Cited by 2 publications

References 0 publications

Clinical features of 11 patients with opsoclonus-myoclonus syndrome diagnosed in an urban pediatric institution in Mexico City: A retrospective study

Clinical features of 11 patients with opsoclonus-myoclonus syndrome diagnosed in an urban pediatric institution in Mexico City: A retrospective study

Neuroblastoma-associated Opsoclonous Myoclonous Ataxia Syndrome: Profile and Outcome Report on 15 Egyptian Patients

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