approach to rhabdomyosarcoma in childhood .pptx

rhabdomyosarcoma

1. Name of the Presenter -Dr Jyoti Sunkara Name of the Centre -Government Cancer Hospital,Aurangabad Diagnosis -Burkitt’s Leukemia/Group C/Stage 4 Date of Presentation -22nd June 2024

2. CASE SUMMARY 12 year old M ale child April 2024 Presenting complaints Abdominal distention Pain in abdomen × 1month Loss of appetite Fatigue

3. On examination: Tachycardia+,Tachypnea+,On O2 P/A-Distended,Mass plapable in Left lumbar region size -10*5cm,Fluid+ Uric acid elevated,LDH-4500U/L

4. March 2024 CT A+P- Homogenously enhancing asymmetric circumferential thickening of max thickness 4.7 cm noted over approx length of 12 cm noted involving jejunum in left lumbar region(jejunal bowel loops contains few fecoliths within)Mass is of size 8.4*12*9.2cm. No obvious luminal narrowing.Proximal bowel non-dilated. Anteriorly-lesion shows loss of fat planes,posteriorly-abutting left psoas muscle,Laterally-reaching upto left lateral abdominal wall,medially displacing bowel loops to periphery. Multiple homogenously enhancing lymph nodes in adjacent mesentery,preaortic,interaortocaval,precaval regions,largest 2.1*2.1cm in preaortic region Likely Small Bowel Lymphoma USG abdomen + Pelvis (March 2024) .Ill-defined heterogenous lesion measuring approximately 11*11*13cm in left lumbar region involving underlying small bowel loops with raised vascularity-S/O neoplastic etiology

5. Investigation s and Diagnosis USG guided biopsy of small bowel lesion Lymphoproliferative disorder HPE High grade B-cell Lymphoma Burkitt Lymphoma Positive for-CD-20,PAX 5,CD10,C-MYC positive,Ki-67-95-100% Negative for CD3,TdT,BCL6,BCL2 IHC Bone Marrow->25% blasts(done after Reduction phase in v/o Respiratory distress) CSF-Negative for malignant cells. Diagnosis-Burkitt’s Leukemia/Stage 4/Group C/CNS negative Staging

6. Course in the hospital: After correcting metabolic abnormalities,Reduction phase was started(LMB96 protocol) USG abdomen- 7.3*13.6*13.8cm lesion in left lumbar region Non-responder Started on Induction with COPADM1 Grade 4 myelosupression,Grade 3 Diarrhea,Grade 4 mucositis ,Febrile Neutropenia -requiring Antibiotics, Oxygen and inotropic support USG adomen- 2.8*7.3*5.1cm lesion left lumbar region with minimal free fluid Once recovered,Induction with COPADM2 was given with 2/3 dose reduction(delay by 5 days) Grade 4 myelosupression,Grade 4 mucositis, Febrile Neutropenia-Antibiotics, inotropic support USG abdomen-Mass like irregular thickening of bowel loops in left lumbar region measuring 3.6*5.5cm noted Recovering from Febrile Neutropenia,Due for CYVE1

7. Question s to the Tumour Board? Experience of panel with Group C Burkitt’s Lymphoma? Reevaluation-whether PET CT or CT has to be done? Does addition of Rituximab has benefits?

8. Thankyou