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Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare malignant neoplasm arising from the neural crest cells of the olfactory epithelium. The optimum treatment for this rare disease is still unclear. Most of the available literature on this rare head and neck tumour is limited to small retrospective series and single institutional reports. We conducted a retrospective study to investigate the clinical profile, treatment outcomes and prognostic factors of patients with ENB treated at a tertiary cancer centre in south India. Patients with a histopathological diagnosis of ENB treated from 2000 to 2019 were included. Patient demographics, tumour characteristics, stage, treatment details and outcome data were identified from medical records. Overall survival (OS) and disease-free survival (DFS) were estimated using the Kaplan-Meier method and the log-rank test was used for comparison. The prognostic factors were identified using Cox regression analysis. Forty-two patients underwent treatment for ENB from 2000 to 2019. Twenty-six patients underwent surgery. Twelve patients received radical radiotherapy (RT) while 24 patients underwent adjuvant radiation. After a median follow-up of 71 months, the estimated OS and DFS at 4 years were 64.4% and 54%, respectively. The estimated 4-year OS for modified Kadish A, B, C and D stages was 75.0%, 90.9%, 56.4% and 0%, respectively. Modified Kadish stage, nodal involvement, orbital invasion, intracranial extension, surgery, RT treatment and use of chemotherapy were significant predictors of OS and DFS in univariate Cox regression analysis. Orbital invasion and RT treatment were significant predictors of DFS in the multivariate analysis as well. However, only RT treatment came out to be a significant predictor for OS in multivariate Cox regression analysis. Surgery is the mainstay of treatment. Adjuvant RT may improve local control and survival in advanced cases. Advanced modified Kadish stage, lymph node involvement and orbital invasion are associated with poor outcomes.
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Lakshmi Haridas K.Introduction Testicular germ cell tumors are rare in India. Despite the advances in chemotherapy, poor-risk testicular nonseminomatous germ cell tumors (NSGCT) remain as a clinical challenge. Various prognostic factors have been described in this rare disease. The Indian data in this regard is scarce. Our study is the first attempt to assess the tumor marker decline with respect to treatment outcome in poor-risk NSGCT in Indian patients. Materials and Methods This retrospective study was done among newly diagnosed poor-risk NSGCT, treated at genitourinary clinic, at our tertiary cancer center during the period 2017 to 2019. The prognostic significance of tumor marker decline in them was correlated with 2-year progression-free survival (PFS) and 2-year overall survival (OS). Statistical Methods The association between two variables were assessed using chi-squared/Fischer's exact test. The PFS and OS were estimated using Kaplan-Meier method and the significance difference between survival curves was tested using log rank test. The risk for survival was estimated using cox regression analysis. A p -value of <0.05 was considered as significant. Results Out of 11 eligible patients, four (36%) had favorable tumor marker decline and seven (64%) had unfavorable decline. The 2-year PFS among favorable and unfavorable decline group were 66.7 and 42.9%, respectively ( p -0.358), and the 2-year OS was 66.7 and 71.4%, respectively ( p -0.974). Teratoma was not found to be a significant factor in our study. Tumors with only beta human chorionic gonadotropin (ßHCG) elevation were observed to have good outcome. Postchemotherapy unresectable residual disease showed a significant trend toward inferior survival, the 2-year PFS was 38 versus 100% ( p -0.188) and the 2-year OS was 62.5 versus 100% ( p -0.334) in patients with and without unresectable residual disease, respectively. Conclusion Majority of our poor-risk NSGCT patients had unfavorable tumor marker decline and progressive events. However, the survival difference was not significant, given the small sample size. Tumors with only ßHCG elevation were observed to have good outcome. Postchemotherapy unresectable residual disease showed a significant trend toward inferior survival.
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BACKGROUND: Data from low- and middle-income countries on tumor lysis syndrome (TLS) in the pediatric population are limited. This study aims to analyze the clinical and biochemical characteristics and treatment outcomes of TLS in children with leukemia/lymphomas in a resource-limited setting. PROCEDURE: Children with intermediate risk (IRD) and high risk (HRD) for developing TLS were retrospectively studied at a tertiary cancer center in India. RESULTS: Over a three-year period, 224 children with acute leukemia/lymphoma having IRD (21.8%, n = 49) and HRD (78.1%, n = 175) were identified. TLS developed in 53.6% (n = 120) cases, of which 75% (n = 90) had laboratory TLS alone. Thirteen children had clinical TLS (C-TLS) at presentation while 17 patients progressed to develop C-TLS. TLS developed in 51% (n = 25) and 54.5% (n = 95) of children with IRD and HRD, respectively. Rasburicase was used in 8.5% (n = 19) cases and five children required hemodialysis. Two children (0.8%) expired during the course of TLS management. Multivariate analysis identified the presence of hyperuricemia as the single significant risk factor for developing TLS. When children in whom a 25% change in biochemical values from the baseline that falls within the normal range were excluded, 21.4% (48/224) cases were identified to have clinically relevant TLS (8% in IRD and 25% in HRD). CONCLUSION: With hydration, supportive care and judicious use of rasburicase, it is feasible to manage TLS efficiently in resource-limited settings. A modification of the TLS definition criteria would help to identify clinically relevant TLS.
