Gnanamani Senguttuvan

and 10 more

Background Infant medulloblastoma (iMBL) are predominantly desmoplastic nodular (DN) or Medulloblastoma with Extensive Nodularity (MBEN) histology, Sonic Hedgehog (SHH) driven. Intensified chemotherapy (intraventricular chemotherapy or high-dose chemotherapy with autologous stem cell rescue) is recommended to compensate the omission of radiation. This study aims to analyse the outcomes of iMBL treated without treatment intensification. Methods This retrospective study was done on 28 patients with iMBL (children with medulloblastoma <3 years at diagnosis or 3-4 years with DN/MBEN) treated between January 2004- April 2024 in a tertiary care institute in Southern India. Results The median age at diagnosis and duration of symptoms were 24.5 months and 1 month, respectively. Vomiting (82%) and ataxia (71.4%) were the common presentations. Ventriculoperitoneal shunt or endoscopic 3 rd ventriculostomy was done in 57% patients. Twenty-four patients (~86%) underwent gross/near total resection. The most common surgical complication was cerebellar mutism (n=6, 21%). Half of the cohort had non-metastatic disease. Sixteen patients had DN/MBEN histology. Only 68%(n=19) opted to continue adjuvant therapy. Chemotherapy protocols used were Baby SFOP, HIT SKK and modified Packers. One fourth (n=7) received radiation (DN/MBEN=3, other histology=4) after 3 years of age. There was no treatment related mortality. The 5-year EFS of patients who received adjuvant treatment, DN/MBEN, non-DN/MBEN histology were 70%, 82.5% and 35.7%, respectively. Conclusions Managing medulloblastoma in infants is challenging in LMIC as significant proportion of families opt against adjuvant treatment. Although traditionally iMBL considered high risk, good outcomes can be achieved with non-intensified therapy in DN/MBEN subgroup.