Synovial sarcoma of kidney in a child: A rare presentation

There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms′ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms′ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction

Real-World Experience of Treating Young Adult Patients with Breast Cancer from a Single Center in Southern India

Abstract Priya Iyer Background Breast cancer in young adults is rare and accounts for 5 to 6% of all cancers in this age group. We conducted the present study to look at the demographic features, clinical presentation, and outcomes in this group of patients treated at our center. Patients and Methods The study included breast cancer patients between the age of 15 and 30 years treated at our institute from January 2009 to December 2016. Data were analyzed retrospectively from case records. Event-free survival (EFS) and overall survival (OS) were calculated using the Kaplan–Meier method. Results Young adult breast cancers were reported in 145 out of 6,000 patients (2.41%) diagnosed with breast cancer in the study period. The median age of the patients was 29 years (range: 21–30 years). Stage I, II, III, and IV was observed in 3.4, 33.7, 46.2, and 16.5% of patients, respectively. The median follow-up was 45 months (range: 1.7–128.1 months). The 5-year EFS and OS for stage I, II, III, and IV was 100, 74.5, 47.9, and 0% and 100, 90.8, 55.1, and 0%, respectively. On univariate analysis, stage of the disease and pregnancy-associated breast cancers were found to have a significant association with decreased EFS and OS (p < 0.001, p = 0.008 and p < 0.001, p = 0.001, respectively). On multivariate analysis, stage of disease and pregnancy-associated breast cancers remained significant predictors of EFS and OS. Conclusion Breast cancers in young adults are rare but need to be diagnosed at an early stage to improve survival. Pregnancy-associated breast cancers need to be managed optimally without delay owing to their aggressive tumor biology

Hepatoblastoma: 16-years’ experience from a tertiary cancer centre in India

Background: Hepatoblastoma is a rare pediatric tumor arising from the liver. The present study was conducted to ascertain the clinical profile and survival outcomes of patients with hepatoblastoma treated at our centre. Methods: We collected the case records of patients with hepatoblastoma treated between January 2000 to December 2016 and analysed the baseline characteristics, treatment details and outcomes. Survival was analysed using Kaplan Meier method. Results: Twenty-seven patients with hepatoblastoma received treatment at our centre during the study period. Median age of the patients was 12 months and 76% were males. The commonest presenting symptom was abdominal mass and the median Alpha Fetoprotein (AFP) level at the time of diagnosis was 40,000 ng/ml. PRETEXT stage II was documented in 11 and III in 11 patients. High risk disease (PRETEXT IV or metastatic disease or portal venous invasion or AFP < 100 ng/ml) was documented in 8/27 (30%) patients. Neoadjuvant chemotherapy (NACT) was given to 23/27 patients and complete surgical resection was possible in 15/23 (65%) after NACT. Infusional cisplatin and doxorubicin (PLADO) was given in 24/27 patients. Liver transplantation was done in 1 patient. The median follow-up was 51 months and the 5-year overall survival for standard risk and high-risk patients was 78.8% and 40% respectively. Conclusion: Patients with standard risk hepatoblastoma have survival outcomes comparable to Western countries, however, outcomes in patients with high risk non-metastatic inoperable disease remains low due to financial constraints in performing liver transplantation. Multimodality treatment including NACT with PLADO based regimens followed by resection is a feasible strategy. Keywords: Hepatoblastoma, PLADO, Pediatric cance

Pediatric Hodgkin Lymphoma Treated at Cancer Institute, Chennai, India: Long-Term Outcome

Purpose: Pediatric Hodgkin lymphoma (HL) is a highly curable malignancy. Outcomes for pediatric HL may vary between developed and developing countries for multiple reasons. This study was conducted to ascertain the outcomes of children with HL at our center and to identify risk factors for recurrent disease. Methods: We analyzed the outcomes of 172 consecutive, previously untreated patients with pediatric HL presenting at our center from 2001 to 2010. Patients were treated with either adriamycin, bleomycin, vinblastine, and dacarbazine or adriamycin, bleomycin, vinblastine, cyclophosphamide, vincristine, prednisone, and procarbazine chemotherapy initially, and radiation to bulky sites or a single site of residual disease when appropriate. Results: The median duration of follow-up was 77 months. The median age of the patients was 10 years; 127 (74%) of the 172 patients were male. The extent of disease was stage I and II in 59% of the patients. B symptoms were present in 32% of the patients, and 27% had bulky disease. The most common histologic subtype was mixed cellularity (45%). The 5-year overall survival (OS) and progression-free survival (PFS) of the entire cohort were 92.9% and 83.1%, respectively. The 5-year OS rates for patients with stage I, II, III, and IV were 96%, 94.7%, 84%, and 69.8%, respectively. On univariate analysis, advanced stage, response on interim radiologic assessment, and presence of B symptoms significantly predicted inferior PFS and OS. On multivariate analysis, only interim radiologic response significantly predicted PFS (P < .001) and OS (P < .001). Conclusion: Overall, the outcomes of patients treated at our center are comparable to those observed in other centers in India and globally

Optimization and Validation of a Harmonized Protocol for Generating Therapeutic-Grade Dendritic Cells in a Randomized Phase II Clinical Trial, Using Two Varied Antigenic Sources

Autologous dendritic cell (DC)-based immunotherapy is a cell-based advanced therapy medicinal product (ATMP) that was first introduced more than three decades ago. In the current study, our objective was to establish a harmonized protocol using two varied antigenic sources and a good manufacturing practice (GMP)-compliant, manual method for generating clinical-grade DCs at a limited-resource academic setting. After obtaining ethical committee-approved informed consent, the recruited patients underwent leukapheresis, and single-batch DC production was carried out. Using responder-independent flow cytometric assays as quality control (QC) criteria, we propose a differentiation and maturation index (DI and MI, respectively), calculated with the QC cut-off and actual scores of each batch for comparison. Changes during cryopreservation and personnel variation were assessed periodically for up to two to three years. Using our harmonized batch production protocol, the average DI was 1.39 and MI was 1.25. Allogenic responder proliferation was observed in all patients, while IFN-gamma secretion, evaluated using flow cytometry, was detected in 10/36 patients and significantly correlated with CD8+ T cell proliferation (p value-0.0002). Tracking the viability and phenotype of cryopreserved MDCs showed a >90% viability for up to three years, while a mature DC phenotype was retained for up to one year. Our results confirm that the manual/semi-automated protocol was simple, consistent, and cost-effective, without the requirement for expensive equipment and without compromising on the quality of the final product