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Management of Neuroblastoma: ICMR Consensus Document

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Abstract

Neuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain. Advanced disease at presentation is common. Diagnosis is established by tumor biopsy and elevated urinary catecholamines. Staging is performed using bone marrow and mIBG scan (FDG-PET/bone scan if mIBG unavailable or non-avid). Age, stage, histopathological grading, MYCN amplification and 11q aberration are important prognostic factors utilized in risk stratification. Low-risk disease including Stage 1 and asymptomatic Stage 2 disease has an excellent prognosis with non-mutilating surgery alone. Perinatal adrenal neuroblastoma may be managed with close observation alone. Intermediate-risk disease consisting largely of unresectable/symptomatic Stage 2/3 disease and infants with Stage 4 disease has good outcome with few cycles of chemotherapy followed by surgical resection. Paraspinal neuroblastomas with cord compression are treated emergently, typically with upfront chemotherapy. Asymptomatic Stage 4S disease may be followed closely without treatment. Organ dysfunction and age below 3 mo would warrant chemotherapy in 4S. High-risk disease includes older children with Stage 4 disease and MYCN amplified tumors. High-risk disease has a suboptimal outcome, though the survival is improving with multimodality therapy including autologous stem cell transplant and immunotherapy. Relapse after multimodality therapy is difficult to salvage. Late presentation, lack of transplant facility, malnutrition and treatment abandonment are additional hurdles for survival in India. The review provides a consensus document on management of NBL for developing countries, including India.

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Acknowledgements

This article is prepared as an outcome of Indian Council of Medical Research (ICMR) Sub-Committee on Pediatric Lymphomas and Solid Tumors coordinated by the Division of Non-Communicable Diseases, ICMR.

Contributions

DB prepared the draft and will be the guarantor. ST performed literature review and contributed to the draft. Oncology inputs were provided by GC, TV, BA, MP, GK, VR and SB. Surgical inputs were provided by SA. Radiotherapy inputs were provided by SL and GKR. SB, GKR and TK coordinated the formation of the ICMR guidelines.

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Authors and Affiliations

  1. Pediatric Hematology Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Deepak Bansal & Sidharth Totadri

  2. Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India

    Girish Chinnaswamy, Tushar Vora, Brijesh Arora & Maya Prasad

  3. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

    Sandeep Agarwala

  4. Department of Pediatric Hematology & Oncology, Rajiv Gandhi Cancer Institute and Research Center, Delhi, India

    Gauri Kapoor

  5. Department of Medical Oncology and Pediatric Oncology, Cancer Institute (W.I.A), Adyar, Chennai, India

    Venkatraman Radhakrishnan

  6. Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai, India

    Siddharth Laskar

  7. NCD Division, Indian Council of Medical Research (ICMR), New Delhi, India

    Tanvir Kaur

  8. Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

    G. K. Rath

  9. Department of Medical Oncology, Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

    Sameer Bakhshi

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  1. Deepak Bansal
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  2. Sidharth Totadri
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  4. Sandeep Agarwala
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  6. Brijesh Arora
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  7. Maya Prasad
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  9. Venkatraman Radhakrishnan
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  10. Siddharth Laskar
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  11. Tanvir Kaur
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  12. G. K. Rath
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  13. Sameer Bakhshi
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Corresponding author

Correspondence to Deepak Bansal.

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ICMR organized the meeting and funded the travel.

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Bansal, D., Totadri, S., Chinnaswamy, G. et al. Management of Neuroblastoma: ICMR Consensus Document. Indian J Pediatr 84, 446–455 (2017). https://doi.org/10.1007/s12098-017-2298-0

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  • DOI: https://doi.org/10.1007/s12098-017-2298-0

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