Neuroblastoma: validation of the INRG classification system in a small series

Elbal, E. Ramos; Miñarro, A. Galera; Riestra, M. E. Llinares; Cortés, M. Bermúdez; Soler, J.L. Fuster

doi:10.1007/s12094-019-02099-7

Cited by 6 publications

(7 citation statements)

References 8 publications

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“…A comparison of our cohort with INRG 2020 report11 (2003–2016) revealed that our cohort showed higher proportion of cases with high-risk features including higher age, high stage (55% vs 46%), high MKI (78% vs 13%), higher unfavourable INPC group (86% vs 36%) and higher proportion of MNA (52% vs 16%), all of which highlight regional variations in clinical presentation of neuroblastoma. Further, in comparison to the smaller series by Ramos Elbal et al ,35 our series showed a higher proportion of MNA and higher proportion of high-risk INRG. The INRG stratification allowed for treatment of the risk groups with recommended protocols and had a significant association with outcome (p=0.02) and overall survival (p=0.0001) in our population too 11 35.…”

Section: Discussioncontrasting

confidence: 67%

“…Further, in comparison to the smaller series by Ramos Elbal et al ,35 our series showed a higher proportion of MNA and higher proportion of high-risk INRG. The INRG stratification allowed for treatment of the risk groups with recommended protocols and had a significant association with outcome (p=0.02) and overall survival (p=0.0001) in our population too 11 35. The overall high mortality rate of 56% in our study is not only due to due to disease related factors but also due to other socio-economic factors leading to treatment abandonment, late referrals, higher stage at presentation and complications like sepsis.…”

Section: Discussioncontrasting

confidence: 67%

“…11q aberration were not studied in this study. Information for 11q aberrations is required for risk stratification of patients with localised unresectable tumours who are <18 months, ≥18 months with differentiating tumours, and for patients with an MS stage and MYCN non-amplified disease 16 35…”

Section: Discussionmentioning

confidence: 99%

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MYCNamplification and International Neuroblastoma Risk Group stratification on fine-needle aspiration biopsy and their correlation to survival in neuroblastoma

et al. 2022

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AimsRisk stratification as per the International Neuroblastoma Risk Group (INRG) stratification is important for management of neuroblastoma. INRG incorporates various parameters including histological category as per the International Neuroblastoma Pathology Classification (INPC) and MYCN amplification, which were evaluated in fine needle aspiration biopsy (FNAB) samples of neuroblastoma patients to ascertain their impact in our population.MethodsThis was a retrospective study including 60 neuroblastoma cases diagnosed on FNAB, staged and stratified by INRG. Mitosis Karyorrhexis Index (MKI), INPC morphological category and MYCN status by fluorescence in situ hybridisation (n=46) were evaluated and correlated to outcome.ResultsThe mean age was 29 months (21 days to 9 years) with 27 and 33 children </≥18 months; male: female ratio of 1.6: 1; INRG stage-30(M), 20(L2), 2(L1) and 2(MS); INRG-36 high-risk, 13 intermediate-risk and 11 low-risk categories, respectively. MKI was high, intermediate and low in 39, 4 and 7 cases, respectively. INPC morphological type included 2 ganglioneuroblastomas and 58 neuroblastomas, graded further as 25 undifferentiated and 33 poorly differentiated tumours. MYCN was amplified in 48% (22/46) cases and correlated with undifferentiated morphology (p=0.01). At a mean follow-up of 469 (7-835) days, 22/50 were disease free and 28/50 had relapsed/died. The overall survival correlated with age (p=0.03), stage (p=0.01), INRG group (p=0.0001) and tumour grade (p=0.036). MYCN status independently did not correlate with age (p=0.5), INRG stage (p=0.2) and overall survival (p=0.4).ConclusionFNAB is a complete modality for diagnosing neuroblastoma and providing all information required for risk stratification as per INRG including MKI, MYCN amplification, INPC category. Our cohort with predominant high-risk neuroblastoma cases highlights regional variation.

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Section: Discussioncontrasting

confidence: 67%

Section: Discussioncontrasting

confidence: 67%

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MYCNamplification and International Neuroblastoma Risk Group stratification on fine-needle aspiration biopsy and their correlation to survival in neuroblastoma

et al. 2022

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“…To overcome these issues, the International Neuroblastoma Staging System was implemented in the early 1990s for staging NB (Kushner et al, 1996;Matthay et al, 2016;Neuroblastoma Treatment PDQR: Health Professional Version, 2002). This staging system was refined further in 2009 by the International Neuroblastoma Risk Group (Monclair et al, 2009;Ramos Elbal et al, 2019). The new staging system consists of L1, L2, M, and MS (Lanza et al, 2019).…”

Section: Staging Of Nb and Biological And Genetic Features Of The Diseasementioning

confidence: 99%

“…The L1 stage is defined as a localized tumor which can be removed by surgery, L2 is defined as localized tumor with image defined risk factors. Stage M is defined as metastasis to other parts of the body, whereas stage MS is metastatic disease present in children younger than 18 months of age (Del Campo Braojos & Donnelly, 2019;Lanza et al, 2019;Monclair et al, 2009;Ramos Elbal et al, 2019). Most of the patients over one year of age are diagnosed with Stage 3 or 4 (Stage MS) disease (Table 1) (Del Campo Braojos & Donnelly, 2019;Lanza et al, 2019;Naranjo et al, 2018).…”

Section: Staging Of Nb and Biological And Genetic Features Of The Diseasementioning

confidence: 99%

The quest to develop an effective therapy for neuroblastoma

Bhoopathi

Padmanabhan

Emdad

et al. 2021

Journal Cellular Physiology

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Neuroblastoma (NB) is a common solid extracranial tumor developing in pediatric populations. NB can spontaneously regress or grow and metastasize displaying resistance to therapy. This tumor is derived from primitive cells, mainly those of the neural crest, in the sympathetic nervous system and usually develops in the adrenal medulla and paraspinal ganglia. Our understanding of the molecular characteristics of human NBs continues to advance documenting abnormalities at the genome, epigenome, and transcriptome levels. The high-risk tumors have MYCN oncogene amplification, and the MYCN transcriptional regulator encoded by the MYCN oncogene is highly expressed in the neural crest. Studies on the biology of NB has enabled a more precise risk stratification strategy and a concomitant reduction in the required treatment in an expanding number of cases worldwide. However, newer treatment strategies are mandated to improve outcomes in pediatric patients who are at high-risk and display relapse. To improve outcomes and survival rates in such high-risk patients, it is necessary to use a multicomponent therapeutic approach. Accuracy in clinical staging of the disease and assessment of the associated risks based on biological, clinical, surgical, and pathological criteria are of paramount importance for prognosis and to effectively plan therapeutic approaches. This review discusses the staging of NB and the biological and genetic features of the disease and several current therapies including targeted delivery of chemotherapy, novel radiation therapy, and immunotherapy for NB.

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