Pediatric germ cell tumor(GCT) with extensive tumor thrombus of inferior vena cava(IVC) presenting as an oncological emergency is extremely rare .Thirteen months female child with sacrococcygeal GCT having IVC and right atrial thrombus presenting with anasarca and respiratory distress is reported. Due to worsening symptoms post initiation of chemotherapy tumour thrombus debulking from right atrium and distal end of IVC was performed on day 9 on emergency basis. Her symptoms improved and after planned chemotherapy tumor excision with coccygectomy could be done. The IVC thrombus remained inoperable due to sclerosis. She remains recurrent-free now 12 months post therapy.

Xeroderma pigmentosum (XP) is an autosomal recessive disorder caused by defect in nucleotide excision repair and is associated with increased incidence of skin cancer. Lymphoblastic leukemia in XP is rare. We report a case of a 11-year-old boy with XP, who developed B – Acute lymphoblastic leukaemia (ALL). He was started on Modified BFM (Berlin-Frankfurt-Münster) ALL Protocol. Tolerance to ALL chemotherapy was good in our XP patient except for the transient derangement of liver function tests during induction, Pseudomonas sepsis and oral mucositis during protocol M and hyperbilirubinemia during maintenance chemotherapy. Though rare, children with XP requires to be investigated for acute leukaemia on development of bicytopenia.

Background: Children with metastatic neuroblastoma have inferior survival despite therapeutic advances. Myeloablative chemotherapy followed by stem cell transplantation, accepted as the current standard of care, is not accessible to patients in many developing countries due to resource constraints. We share our experience of treating metastatic neuroblastoma in a non-transplant facility with conventional chemotherapy, surgery, and radiotherapy. Method - Retrospective study of children 1-14years of age treated for metastatic neuroblastoma in our center from January 2008 to December 2017 Results – Eighty-nine patients with metastatic neuroblastoma received treatment. Mean age was 3.5years and male:female ratio was 1.1:1. The commonest primary site was suprarenal(55%) and commonest site of metastasis was bone marrow(76%). 40% patients had multiple metastatic sites. Mean baseline LDH was 3724 U/L(range303-16609 U/L) and most(65%) patients had LDH>750U/L.53 patients(59.6%)had good response to chemotherapy as evidenced by clearance of metastatic disease, but out of them, 43 patients (81%) progressed subsequently. 26 patients underwent surgery and 12 patients received maintenance therapy. 74 patients(86%) developed recurrence and all but one died. Median time to recurrence and death were 9months(range 0-120months) and 10months(range 1-123months) respectively. At a median follow-up of 72months(range15-135months), 16 patients are alive, with 5-year disease-free survival and overall survival of 17.6% and 18.4% respectively. Age, baseline LDH, chemotherapy regimen and response to treatment affected survival. Conclusion: Outcome of non-infant metastatic neuroblastoma remains dismal in a non-transplant setting. Younger age, lower baseline LDH and good response to chemotherapy appear to confer survival advantage, and may be used for risk-stratification in developing countries.

Background: Few studies have looked into the impact of hypoglobulinaemia on infectious complications in childhood acute lymphoblastic leukemia (ALL). We conducted this prospective study to analyse the profile of severe infections during maintenance chemotherapy in Indian children and their correlation with serum immunoglobulin levels. Methodology: Children ≤14 years with ALL on maintenance chemotherapy were recruited and serum immunoglobulin levels were measured at the time-of-recruitment in this study conducted between 1st April 2018 and 31st March 2019. Children were followed up for severe infection for a period of 6 months or till completion of treatment whichever was later. Statistical analysis was done to find out risk factors of severe infection including serum immunoglobulin status. Results: We recruited 199 children undergoing maintenance chemotherapy (58, 52, 47, and 42 children in 0-6, 7-12, 13-18 and 19-24 months of maintenance period) and followed them up for a mean (SD) 9.7(2.961) months. 56.8%, 80.4%, and 86.4% children had hypo-IgG, hypo-IgA, and hypo-IgM at the time-of-recruitment. Ninety-one (45.7%) children developed 147 episodes of severe infections of which 54 (59.3%) were respiratory. Univariate analysis showed younger age, female gender and normal IgG group had significantly increased risk of severe infection (P=0.024, 0.007, 0.049, respectively), in multivariate analysis female gender had significantly increased risk of severe infection (P=0.025). Conclusion: Significant proportion of Indian children on ALL maintenance chemotherapy developed severe infection and hypoglobulinaemia. However, hypoglobulinaemia did not significantly increase the risk of severe infection. Younger children and female gender had significantly increased risk of severe infection during maintenance.