A 2 year old boy presents with abnormal pigmentation around eyes with abnormal eye movements. It is noted that child has spontaneous bursts of non rhythmic conjugate eye movements in various directions. He also suffers from hypotonia and myoclonus. Fuher physical examination reveals an abdominal mass. CT scan is done with the following findings: Which of the following drug is approved for the above clinical scenario?
Correct Answer: Dinutuximab
Description: Image shows Neuroblastoma. (a) raccoon eye (left eyelid) in a child with metastatic disease; (b) CT abdomen : a suprarenal mass s/o neuroblastorna. Explanation : Ipilumumab: melanoma Atezolizumab: NSCLC, urothelial cancer Dinutuximab: Neuroblastoma Olarutumab: Soft tissue sarcoma Non rhythmic conjugate eye movements associated with hypotonia and myoclonus suggest the diagnosis of opsoclonus-myoclonus syndrome. This is a rare para-neoplastic syndrome associated with neuroblastoma in children. Neuroblastoma is Most common malignant tumour of infancy Most common extracranial solid tumour of childhood Most common abdominal malignancy in childhood Most common site of neuroblastoma is Adrenal Medulla. Clinically it presents as a retroperitoneal mass displacing the kidney leading to Drooping Lily Sign on imaging , anorexia and weight loss. One of the rare paraneoplastic manifestation is opsoclonus-myoclonus syndrome. Histopathologically , it presents as a small round blue cell tumour with Homer wright pseudorosettes and it tests positive for neuron specific enolase. Rearrangement or deletion of sho arm of chromosome 1 is seen in 80% cases. Amplification of N-myc gene impas poor prognosis. Tumour is also associated with elevated levels of catecholamines in 90% of cases. Shimda classification is useful for determining prognosis of neuroblastoma.
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