title: Management of Neuroblastoma: ICMR Consensus Document.
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.
authors.affiliation: Pediatric Hematology Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India. deepakbansaldr@gmail.com.
authors.forename: Deepak
authors.identifier: http://orcid.org/0000-0002-1009-7649
authors.initials: D
authors.lastname: Bansal
authors.affiliation: Pediatric Hematology Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Sidharth
authors.initials: S
authors.lastname: Totadri
authors.affiliation: Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
authors.forename: Girish
authors.initials: G
authors.lastname: Chinnaswamy
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sandeep
authors.initials: S
authors.lastname: Agarwala
authors.affiliation: Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
authors.forename: Tushar
authors.initials: T
authors.lastname: Vora
authors.affiliation: Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
authors.forename: Brijesh
authors.initials: B
authors.lastname: Arora
authors.affiliation: Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
authors.forename: Maya
authors.initials: M
authors.lastname: Prasad
authors.affiliation: Department of Pediatric Hematology & Oncology, Rajiv Gandhi Cancer Institute and Research Center, Delhi, India.
authors.forename: Gauri
authors.initials: G
authors.lastname: Kapoor
authors.affiliation: Department of Medical Oncology and Pediatric Oncology, Cancer Institute (W.I.A), Adyar, Chennai, India.
authors.forename: Venkatraman
authors.initials: V
authors.lastname: Radhakrishnan
authors.affiliation: Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
authors.forename: Siddharth
authors.initials: S
authors.lastname: Laskar
authors.affiliation: NCD Division, Indian Council of Medical Research (ICMR), New Delhi, India.
authors.forename: Tanvir
authors.initials: T
authors.lastname: Kaur
authors.affiliation: Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: G K
authors.initials: GK
authors.lastname: Rath
authors.affiliation: Department of Medical Oncology, Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sameer
authors.initials: S
authors.lastname: Bakhshi
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D007194:India; D009423:Nervous System Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D009447:Neuroblastoma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*
keywords: Autologous; Chemotherapy; Guidelines; ICMR; Neuroblastoma; Treatment