India Cooperative Neuroblastoma Study: Non-high risk (ICONS-NHR-23).
● Principal Investigator: Dr Yamini Krishnan
● Primary objective is to utilize a uniform staging system, that includes age, stage, pathology and limited genomic testing, to accurately risk stratify patients with neuroblastoma in India, and to document >80% 3-year overall survival outcomes for patients with the following:
-Very low-risk neuroblastoma treated with observation alone.
-Low-risk neuroblastoma treated with observation or surgery only.
-Low-risk neuroblastoma and life-threatening symptoms treated with chemotherapy.
-Intermediate-risk neuroblastoma treated with chemotherapy and surgery
● All newly diagnosed patients with confirmed low-risk and intermittent-risk neuroblastoma, seen at participating institutions are eligible for this study.
● All patients and/or their parents or legal guardians must sign a written informed consent with age-appropriate child assent.
● Patients who are high risk i.e. they have tumors with MYCN amplification or have metastatic disease and are over 12 months of age or are 12-18 months of age with documented SCA, are excluded from this protocol.
● Patients with ganglioneuroma are excluded.
● Absent informed consent and age-appropriate assent.
● In the case of children who have received chemotherapy for LTS there is no documentation of the chemotherapy received or the child has received more than 2 cycles of chemotherapy.
● 3-year overall survival outcome for children with low risk and intermediate risk neuroblastoma.
● To implement a uniform risk stratification.
● To determine the progression free survival.
● To establish an INPHOG neuroblastoma surgical tumour board.
Neuroblastoma (NB) is the commonest extracranial solid tumour in children. treatment in India poses challenges because of the relatively low number of patients an individual oncologist might see with subsequent variations in treatment. Recently, Kanwar and colleagues presented the InPHOG 01 neuroblastoma registration study resultsfrom India . 322 children were diagnosed with NB from 2016 to 2021 in 14 centres across India. The staging included PET-CT (177/322), MYCN (233/322), and pathology (INPC). Histopathology was favorable and unfavorable in 107 and 187 children respectively. INRG staging demonstrated that almost half of the patients were classified as L1 (n=41). L2 (n=95) and MS (n=15), with M constituting the remainder. A total of 190 patients underwent biopsy, 50 surgery, and 228 received chemotherapy, with 3-year event free survival (EFS) for L1=84.8%, L2=66.4%, and MS= 46.7%. It is with this background that the InPHOG NB 23-02 study is being planned.Primary objective is to utilize a uniform staging system, that includes age, stage, pathology and limited genomic testing, to accurately risk stratify patients with neuroblastoma in India, and to document >80% 3-year overall survival outcomes for children with low risk and intermediate Risk neuroblastoma..This study is open to newly diagnosed neuroblastoma patients at InPHOG partner institutions, with low or intermediate risk disease determined by age, stage, and MYCN status. Infants under 6 months with adrenal mass < 5cm in diameter and no spread evidence are eligible. Patients with prior emergent chemotherapy due to life-threatening symptoms are eligible, but documentation is required. Written consent and institutional ethics must be met.This protocol excludes high-risk patients with MYCN amplification, metastatic disease, over 12 months of age, or those with ganglioneuroma, and children with LTS chemotherapy without documented or multiple cycles. Children will be given a uniform protocol at all centres and followed up for overall survival.