Mulibrey-Nanism (Muscle-liver-brain-eye nanism= dwarfism) is a rare syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. Wilms tumor has been reported to be associated with this syndrome. Here we report a 2-year-old boy with Wilms tumor and Mulibrey-Nanism and review the published similar cases. Our patient had associated atrial septal defect, atrial flutter and an episode of cardiac arrest along with other features of Mulibrey Nanism. We managed him successfully with chemotherapy and surgery. A total of 14 cases (including present case) of Wilms tumor have been reported in Mulibrey-Nanism. Longest survivor has been 15-year-old.

Background- Superior Vena Cava Syndrome (SVCS) is a life-threatening oncological emergency affecting children with T-cell Acute lymphoblastic Leukemia (T-ALL)/ T-Lymphoblastic Lymphoma (T-LBL). This can prove fatal if not managed appropriately and promptly. Method- We describe critical care needs and outcome of T-ALL/T-LBL patients managed in our unit from 1 May 2016 to 31 March 2021. Result- Twenty-three of the 120 pediatric ALL/LBL patients (19%) managed in our unit had T-ALL/T-LBL. Eleven (48%) patients presented with SVCS. All presented with cough and dyspnea. Chest X-Ray showed mediastinal widening in all patients. Flow cytometry in various body fluids could reach prompt diagnosis in 90% patients and mass biopsy was performed in only 1 patient. Eight patients required Pediatric Intensive Care Unit support. All 11 patients were started on corticosteroids soon after diagnosis of SVCS. The median time to symptom relief was 4 days and that for significant improvement on X-ray was 7days. At a median follow up of 23-months (6-63 months) overall survival and event-free survival was 75%. Conclusion- T-ALL/T-LBL patients with SVCS with good critical care can have improved outcomes. Prompt therapeutic interventions like pericardiocentesis can be life-saving. Flow cytometry can be a handy tool for quick diagnosis and help avoid invasive procedures in sick patients.