A 3-year-old boy is found to have 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 and following clinical finding. CT scan revealed the following finding. Which of the following is false regarding the patient's condition?
Image: - (a) Raccoon eye (left eyelid) in a child with metastatic disease (b) CT abdomen: a suprarenal mass s/o Neuroblastoma. Non rhythmic conjugate eye movements associated with hypotonia and myoclonus point towards 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. 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.
The most common site of tumor is adrenal medulla.
90% of tumours are associated with elevated production of catecholamines.
Rearrangement or deletion of sho arm of chromosome 1 is seen in 80% cases.
Immunohistochemical detection of chromogranin is useful for diagnosis.
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