RESUMEN
A nine years old male child with neurofibromatosis type I and Ganglioneuroblastoma of right adrenal is reported. At presentation, imaging studies, bone marrow examination and biopsy revealed stage III tumour. In view of the large size which was 15 x 10 cms on abdominal ultrasound, 6 cycles of chemotherapy were given. This considerably reduced the size to 8.6x7 cms. At this stage surgical excision was decided. Due to extensive local invasion around inferior vena cava and aorta, approximately 90% of tumour could be excised. One hour following surgery the child went into shock with sudden gush of 500 ml of fresh blood into the drainage bag. Intensive resuscitative measures were carried out with infusion of 2 pints of blood. Plan was to reopen the child after stabilization but he could not be revived and expired. Histopathology of tumour tissue confirmed ganglioneuroblastoma