Watch and See Strategy in Selected Neuroblastoma Case Scenarios: Success and Limitations

Fawzy, Mohamed; Zomor, Hossam El; Menawi, Salma El; Elkinaai, Naglaa; Ahmed, Gehad; Refaat, Amal; Elahmadawy, Mai Amr; Zaghloul, Mohamed S.

doi:10.1097/mph.0000000000001421

“…Same study showed that these tumors do not always have favorable characteristics, and that MYCN amplification continues to be associated with a poor prognosis whenever encountered. 4,19 Despite the fact that not all OMAS patients must show all 3 neurological components at presentation, still the majority of patients (10/15; 67%) had a full-blown picture versus a combination of only 2 neurological symptoms in the other 5/15 (33%) which was exactly found by CCG in their OMAS study between 1980 and 1994, where the majority of their patients showed all 3 criteria, while only 38% experienced 1 or 2 manifestations. 4 All types of onset sequence relation between OMAS and NBL were reported in the present study cohort, where OMAS preceded the clinical detection of the tumor within 3 months to 2 years in only 5 patients (33%) versus 6 patients (40%) having OMAS diagnosed concurrently with tumor diagnosis and 4 patients (27%) developed OMAS symptoms within 6 months to 1.5 years after diagnosis of NBL.…”

Section: Discussionmentioning

confidence: 88%

“…However, absence of MYCN gene amplification among all study patients who showed completely normal pattern was very close to the CCG results that reported only 1 of 21 OMAS patients had MYCN gene amplification. Same study showed that these tumors do not always have favorable characteristics, and that MYCN amplification continues to be associated with a poor prognosis whenever encountered 4,19…”

Section: Discussionmentioning

confidence: 99%

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

Elzomor

¹

,

Menawi

²

,

ElAwady

³

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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“…4 Here, most tumors can be treated with active observation only with modest outcome(s) anticipated including stage 4S (INRG stage MS) neuroblastoma mortality ranging from 0 to 19%. 8,[15][16][17][18] Surgery with or without chemotherapy yielded excellent outcome(s) in stage 4S (INRG stage MS) neuroblastoma according to the quality of published literature reviewed here in this systematic review. Patients suitable for surgical resection of the offending primary tumor only had the best outcome(s).…”

Section: Discussionmentioning

confidence: 90%

Untitled

2021

Eur J Pediatr Surg

0

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Introduction The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma. Materials and Methods Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author. Results The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fatality rate. Management included observation only (201 patients, 8.5% mortality), surgical resection of primary tumor only (153 patients, 6.5% mortality), chemotherapy only (186 patients, 21% mortality), radiotherapy (5 deaths, 33% mortality), chemotherapy with surgery (160 patients, 10% mortality), surgery with radiotherapy (21 patients, 19% mortality), radiotherapy with chemotherapy (42 patients, 29% mortality), and surgery with chemotherapy and radiotherapy (27 patients, 33% mortality). Conclusion There is a significant mortality observed in stage 4S/MS neuroblastoma infants with a dismal outcome observed in those patients with MYCN amplification and 1p/11q deletion. Those patients suitably amenable for conservative management or surgery to excise the primary tumor carry the best prognosis.

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“…4 Here, most tumors can be treated with active observation only with modest outcome(s) anticipated including stage 4S (INRG stage MS) neuroblastoma mortality ranging from 0 to 19%. 8,[15][16][17][18] Surgery with or without chemotherapy yielded excellent outcome(s) in stage 4S (INRG stage MS) neuroblastoma according to the quality of published literature reviewed here in this systematic review. Patients suitable for surgical resection of the offending primary tumor only had the best outcome(s).…”

Section: Discussionmentioning

confidence: 99%

Stage 4S Neuroblastoma: What Are the Outcomes? A Systematic Review of Published Studies

Raitio

¹

,

Rice

²

,

Mullassery

³

et al. 2020

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Introduction The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma. Materials and Methods Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author. Results The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fatality rate. Management included observation only (201 patients, 8.5% mortality), surgical resection of primary tumor only (153 patients, 6.5% mortality), chemotherapy only (186 patients, 21% mortality), radiotherapy (5 deaths, 33% mortality), chemotherapy with surgery (160 patients, 10% mortality), surgery with radiotherapy (21 patients, 19% mortality), radiotherapy with chemotherapy (42 patients, 29% mortality), and surgery with chemotherapy and radiotherapy (27 patients, 33% mortality). Conclusion There is a significant mortality observed in stage 4S/MS neuroblastoma infants with a dismal outcome observed in those patients with MYCN amplification and 1p/11q deletion. Those patients suitably amenable for conservative management or surgery to excise the primary tumor carry the best prognosis.

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Watch and See Strategy in Selected Neuroblastoma Case Scenarios: Success and Limitations

Cited by 6 publications

References 16 publications

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

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

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Stage 4S Neuroblastoma: What Are the Outcomes? A Systematic Review of Published Studies

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