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Purpose Metabolic syndrome (MetSyn) is an important late effect of childhood cancer. The combination of rising obesity and high prevalence of under-nutrition at diagnosis makes this a unique population to study in LMIC (lower middle-income countries). Methods Children ≤ 18 years of age at cancer diagnosis, in a single center in a LMIC, who were disease free and had completed treatment at least 2 years prior to study were included. MetSyn was defined using International Federation for Diabetes criteria for Asian Indians. Logistic regression analyses were carried out to evaluate the influence of various risk factors, including delta BMI (increase in body mass index from diagnosis to evaluation), on MetSyn. Results A high prevalence of MetSyn (12.2%), central obesity (33%), and dyslipidemia (61.8%) were found in a cohort of 500 Asian Indian childhood cancer survivors (CCS) at a median follow-up age of 17 years. Multivariable analysis revealed older age at diagnosis ≥ 10 years, OR 2.9 (1.6–5); longer survival duration ≥ 10 years, OR 2.2 (1.3–3.8); high BMI at diagnosis, OR 3.2 (1.5–6.9); and large delta BMI ≥ 50, OR 3.15(1.7–5.9) to be independent predictors of MetSyn. Patients who were underweight or normal at diagnosis with large delta BMI ≥ 50 had very high odds (OR, 12.5, 1.7–92) of developing MetSyn compared to those with lower delta BMI. Conclusions and implications for cancer survivors A high prevalence of MetSyn was observed in CCS with early age at onset. Timely screening and early intervention are proven to be beneficial and delta BMI could be a useful screening tool for LMIC.

Sex disparity and its determinants in childhood cancer in India remain unexplored, with scarce information available through summary statistics of cancer registries. This study analysed the degree of sex bias in childhood cancer in India and its clinical and demographical associations. In this retrospective, multicentre cohort study, we collected individual data of children (aged 0–19 years) with cancer extracted from the hospital-based records of three cancer centres in India between Jan 1, 2005, and Dec 31, 2019, and two population-based cancer registries (PBCRs; Delhi [between Jan 1, 2005, and Dec 31, 2014] and Madras Metropolitan Tumour Registry [between Jan 1, 2005, and Dec 31, 2017]). We extracted data on age, sex, and confirmed diagnosis of malignancy (according to the International Classification of Diseases-10 coding),and excluded participants if they were without a recorded diagnosis, had a benign diagnosis, had missing sex information, resided outside of India, or were a donor for haematopoietic stem cell transplantation (HSCT). The primary outcome was the male-to-female incidence rate ratio (MF-IRR) in the two PBCRs and the male-to-female ratios (MFR) from the hospital-based and the HSCT data. For PBCR data, MF-IRR was estimated by dividing the MFR by the total population at risk. MFR was analysed for patients seeking treatment at the cancer centres and for those undergoing HSCT. Logistic regression analyses were done to explore the association of clinical and demographical variables with sex of the patients seeking treatment and those undergoing HSCT in hospital-based data and multivariable analyses were done to determine independent sociodemographic predictors of sex bias. Annual time trends of MFR and MF-IRR during the 15-year study period were ascertained by time series regression analyses. We included 11 375 children from PBCRs in the study. 26 891 children from hospital-based records were screened, and data from 22 893 (85·1%) were included (including 514 who underwent HSCT). Residence details were missing for 257 (1·1%) of 22 893 patients from hospital-based records. The crude MFR of children at diagnosis was in favour of boys: 2·00 (95% CI 1·92–2·09) in the Delhi PBCR and 1·44 (1·32–1·57) in Madras Metropolitan Tumour Registry. The MF-IRRs for cancer diagnosis were also skewed in favour of boys in both PBCRs (Delhi 1·69 [95% CI 1·61–1·76]; Madras Metropolitan Tumour Registry 1·37 [1·26–1·49]). The MFR for children seeking treatment from hospital-based records was 2·06 (95% CI 2·00–2·12) in favour of boys. In subgroup analyses, the proportion of boys seeking treatment was higher in northern India than southern India (p<0·0001); in private centres than in centres providing subsidised treatment (p<0·0001); in patients with haematological malignancies than those with solid malignancies (p<0·0001); in those residing 100 km or further from the hospital than those within 100 km of a hospital (p<0·0001); and those living in rural areas than those living in urban areas (p=0·0006). The MFR of 514 children who underwent HSCT was 2·81 (95% CI 2·32–3·43) in favour of boys. Time trend analysis showed that MFR did not show any significant annual change in either the overall cohort or in any of the individual centres for hospital-based data; however, the analysis did show a declining MF-IRR in the Delhi PBCR from 2005 to 2014 (p=0·031). The sex ratio for childhood cancer in India has a bias towards boys at the level of diagnosis, which is more pronounced in northern India and in situations demanding greater financial commitment. Addressing societal sex bias and enhancing affordable health care for girls should be pursued simultaneously in India. None. For the Hindi translation of the abstract see Supplementary Materials section.

ObjectiveTo describe the prevalence of obesity and sarcopenia among survivors of childhood acute lymphoblastic leukemia (ALL) using DEXA scan, and study associated risk factors.MethodsThis case control study was conducted between July, 2013 and June, 2014 at a tertiary care cancer centre in India. Study participants included 65 survivors of childhood ALL who were <18 years of age at diagnosis, treated between years 1996 and 2008, and were at least two years since completion of therapy. The controls included 50 matched siblings. Dual energy X-ray absorptiometry (DEXA) was used to study the body composition (body fat percentage, BF% and lean body mass, LBM) of the participants and controls. McCarthy’s body fat reference data were applied and logistic regression analysis was used to study various risk factors.ResultsAt a median (range) follow-up of 5 (7.2–17.2) years, BF% (DEXA) identified a significantly higher prevalence of obesity of 21.5% (14/65) and sarcopenic obesity (14%) among survivors as compared to the controls (0/50, P<0.001), while the prevalence of sarcopenia as detected by LBM was similar at 60% (39/65) and 56% (28/50), respectively. On multivariate analysis, age at evaluation, high-risk disease and cranial irradiation were independently associated with high likelihood of obesity, while none of the factors predicted sarcopenia.ConclusionHigh prevalence of obesity and sarcopenic obesity were observed among survivors of childhood ALL.

We report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonance imaging (MRI) orbit revealed a lobulated heterogeneously enhancing right retroocular mass extending up to the orbital apex, displacing the optic nerve and eroding the medial orbital wall. Biopsy of the lesion revealed pure YST histology. Serum alpha-fetoprotein (AFP) was markedly raised at 76900 ng/mL. She was started on infant bleomycin etoposide cisplatin (BEP) chemotherapy protocol. There was a good clinical and radiological response. A high index of malignancy is required in young children presenting with orbital proptosis. A multidisciplinary approach and early intervention are essential to save both vision and life.

Molecular abnormalities in leukemic cells are important determinants of risk stratification in Pediatric acute lymphoblastic leukemia (ALL). TCF3-PBX1 fusion is one of the common aberrations in ALL with doubtful prognostic significance. Therefore, aim of our study is to revisit the clinical characteristics and outcome of this abnormality in children with ALL treated at our institute.Demographic, Clinical and treatment related characteristics of 539 newly diagnosed ALL patients from January 2009 and December 2018, < 18 years of age treated on BFM-95 protocol, was abstracted from the medical records. Clinical characteristics and outcome of children with and without TCF3-PBX1 fusion was compared.Incidence of TCF3-PBX1 fusion was observed in 24/539(4.4%) patients with a median age of 4 years (range 1–17). None of the patients in TCF3-PBX1 group had CNS or testicular disease at presentation. Day -8 prednisolone response and morphological remission at the end of induction was similar in both study groups. 5-year overall and event free survival for those with and without fusion was 75%, 70.1% and 79.5%, 69.5% respectively.The incidence of TCF3-PBX1 fusion in the present study was 4.4% and it does not have an independent prognostic significance.

Surgical resection with wide margins is pivotal for sarcoma treatment but achieving the same for fibular sarcomas is a surgical challenge. Thus, we decided to evaluate our own institutional database of primary fibular sarcomas for surgical treatment, margins and pattern of relapse. From July 2014 to October 2018, we identified fourteen patients with histologically confirmed fibular sarcomas. Limb salvage surgery (LSS) was performed in thirteen patients included in our study. One patient treated with definitive radiotherapy was excluded from final survival and functional analyses. The proximal third fibula was the most common site of involvement (85.7%). Osteosarcoma was the histological diagnosis in eight (57.1%) and Ewing’s in the remaining six (42.9%). All patients with proximal fibular tumours underwent Malawer type II resection. Margins were reported as free in twelve and involved in one case. The mean follow-up period was 37.15 months. In the operated group (n = 13), distant relapse occurred in 3 patients, combined relapse in 1 patient and 10 patients are alive and disease free until the last follow-up. The Kaplan-Meier survival analyses revealed the EFS (event-free survival-local/distant relapse) probability as 72.7% at 24 months and 53% at the end of 42 months. The OS (overall survival) probability at 24 months was 75.5% and 57.5% at the end of 42 months. Although it is difficult to achieve conventional wide margins in fibular sarcomas, our results suggest no increased incidence of local recurrence rates as compared to sarcomas at other common sites as reported in literature. Our series helps in understanding site-specific behaviour of sarcomas while contributing to the available data.

Abstract Background: Continuum of care is an important concern for childhood cancer survivors. Studies from high-income countries indicate a significant development in services to these survivors. Similar information is unavailable from India. Methods: An online survey form was developed and sent to 86 centers. Data were collected over a 6-month period in 2017. Results: Fifty nine centers responded (44.1% private sector, 33.9% public, and 22.0% charitable trust). The services are mainly provided (91%) within routine oncology clinics. There is no upper age limit (61%) or time period limit (63%) for follow-up at most of the centers. The major barriers for follow-up are distance, lack of knowledge, lack of adequate facilities, and patient priority for follow-up. Conclusion: This survey provides baseline information on current service provided to childhood cancer survivors in India. There is a need to inform, educate, and sensitize the survivor and their family as well as improving services.

Acute megakaryoblastic leukemia (AMKL) is a rare disease accounting for 7%–10% of acute myeloid leukemia (AML) in children. It is uncommon in children without Down syndrome (DS). Orbital chloroma is usually associated with AML M2, M4, and M5. Herein, we report the case of a 22-month-old female who was diagnosed to have AMKL with orbital chloromas and without DS. Morphology and the initial panel of immunophenotyping were inconclusive and the presence of orbital chloromas added to the ambiguity. The presence of CD61 expression and marrow fibrosis supported by hyperdiploidy helped us clinch the diagnosis. Hence, comprehensive analysis of morphology, immunophenotyping, and cytogenetics is warranted to make an accurate diagnosis of AMKL.