Vinay Munikoty

and 8 more

Background. Iron overload may contribute to complications in childhood cancer survivors. Methodology. Patients treated for hematological malignancy, ≥6 months from the end of therapy, who had received ≥5 red-cell transfusions were enrolled in the cross-sectional study. Iron-overload was estimated by serum ferritin (SF) and T2*MRI. Results. Forty-five survivors were enrolled among 431 treated for hematological malignancies. The median age at diagnosis was 7-years. The median number of red-cell units transfused was 8 (IQR 7, 10). The median duration from the end of treatment was 15 months (IQR 8.75, 25). An elevated SF (>1000 ng/ml), elevated liver iron concentration (LIC) and myocardial iron concentration (MIC) were observed in 5 (11.1%), 20 (45.4%), and 2 (4.5%) patients, respectively. All survivors with SF >1000 ng/ml had elevated LIC. The LIC correlated with SF (p<0.001). MIC lacked correlation with SF or LIC. The number of red-cell units transfused and duration from last transfusion correlated with SF (p=0.001, 0.002) and LIC (p=0.012, 0.005). SF >500 ng/ml predicted elevated LIC with sensitivity/specificity of 80%/79%. A cut-off of 8 units of red-cells predicted elevated LIC with sensitivity/specificity of 95%/49.8%. Conclusions. Iron overload in survivors of hematological malignancies who had received ≥5 red-cell transfusions, estimated by SF, LIC, and MIC was 11.1%, 45.4%, and 4.5%. We suggest screening by SF for survivors who have received >8 transfusions. If SF is 500-1000 ng/ml, a T2*MRI is useful for estimating LIC. A T2*MRI can be avoided if SF exceeds 1000 ng/ml, as LIC will be expected to be elevated.

Renu Madan

and 9 more

Background: Series on radiotherapy (RT) practice in pediatric malignancies are limited in India as only few centers practice pediatric RT, particularly under anesthesia. We aimed to study the clinical profile of pediatric cancer patients treated with RT and to assess various aspects of pediatric RT under anaesthesia. Procedure: The data was prospectively maintained in Microsoft Excel spreadsheets. Pediatric cancer patients aged 0–14 years, registered in the department between February 1, 2019, and July 30, 2021were analyzed. Results: A total of 193 pediatric cancer patients (non central nervous system) received radiation during above mentioned period. Median age at presentation was 5.2 years (range, 9 months to 14 years) with a male to female ratio of 1.8:1. Maximum population was in 0-4 years of age group (52.8%) followed by 5-9 years (29.5%) and ≥ 10 years (17.6%). Most common sites for RT included bone and soft tissue tumors (BST), retinoblastoma, wilms tumor, neuroblastoma, and hematological malignancies. Overall, BST constituted the most common malignancy among all age groups, followed by renal tumors in the 0-4 years and hematological malignancies in 5-14 years. One hundred and seventy-nine (92.7%) patients received RT with curative intent, while 14 (7.3%) patients received palliative RT. Thirty patients needed anaesthesia for RT. Thirty-four patients required RT interruption due to toxicities with a median gap of 7 days. Conclusions: RT is an important aspect of multi-disciplinary care in pediatric cancers. Establishing burden of pediatric cancer patients in RT department may help in resource development and prioritisation.
Background: ABVD regimen for Hodgkin lymphoma (HL), disfavoured in high-income countries, is popular in low-middle-income countries (LMIC). The feasibility/safety data for ‘non-ABVD’ protocols from LMIC is limited. Procedure: The retrospective study was conducted in a single center in India. Euronet-PHL-C1-based protocol was administered during 2010-19. A PET-CT was performed at diagnosis and following OEPA-course-2. Radiotherapy was administered for inadequate PET-response. Results: During the 10-year-period, 143 patients with HL were treated. The mean-age was 7.8±2.5 years. Bulky-disease was observed in 82 (59%). Treatment-abandonment was recorded in 13 (9.1%). The median follow-up duration was 46.4 months. An inadequate PET-response was observed in 41/118 (34.7%). Radiotherapy was administered to 23/41 (56.1%). There was a protocol violation of replacing radiotherapy in 12 (29.3%) patients with 2-courses of COPDAC. Sixty-nine episodes of febrile-neutropenia were observed in 54 patients. TRM was observed in 7 (5.3%). The majority of episodes of febrile-neutropenia (61%) and TRM (86%) were following OEPA-course-1. The 4-year overall-survival (OS) and event-free survival (EFS) were 93.5±2.2% and 86.2±3.4%, respectively. Nine (6.3%) patients relapsed. The survival compared favorably with 5-year-EFS (77.7%) of patients who received ABVD/COPP in the center in the past. Bulky-disease lacked association with inadequate PET-response (p=0.800) or relapse (p=1.000). Conclusions: OEPA/COPDAC regimen and response assessment by PET-CT permitted therapy reduction, including radiotherapy. The survival (4-year OS: 93.5±2.2%) was excellent, with a low relapse (6.3%). Febrile neutropenia and resultant TRM (5.3%) are concerning and occurred frequently following OEPA-course-1. The support system for managing febrile neutropenia should be optimized for administering OEPA in LMIC.

Radhika Srinivasan

and 5 more

Objective:  Anaplastic Lymphoma Kinase ( ALK) gene gain-of-function point mutation leading to its overexpression has recently been identified and targeted in neuroblastoma (NB). We evaluated ALK gene mutation and its protein expression in cases of NB on fine needle aspiration biopsy (FNAB). Material and Methods: FNAB diagnosed cases of NB (n=56) were evaluated with cell blocks for MYCN amplification and ALK protein expression by Fluorescence in-situ hybridization and immunocytochemistry respectively. MGG stained smears (n=22) were used for Next generation sequencing (NGS) analysis using the Cancer Hotspot panel (version2) on Ion Torrent S5 platform. Staging and risk assignment as per International Neuroblastoma Risk Group (INRG) was performed and managed. All the parameters were correlated with overall survival. Results: ALK protein showed cytoplasmic expression in 65% of cases and did not correlate with MYCN amplification (p=0.35), INRG groups (p=0.52), and overall survival (p=0.2); however, ALK+ poorly differentiated NB showed better prognosis (p=0.02). ALK negativity was associated with poor outcome by Cox proportional hazard model (hazard ratio=2.36). ALK gene, exon 23 missense mutations (F1174L) were seen in 2/21 cases with an allele frequency of 8% and 54%. Both these cases showed ALK protein expression and died of disease within 1 and 17 months respectively. A novel IDH1 exon 4 mutation was also detected. Conclusion:  ALK expression is a promising prognostic as well as a predictive marker in advanced NB along with traditional prognostic parameters. FNAB smears are suitable for NGS and ALK gene mutation confers a poor prognosis.

Richa Jain

and 10 more

Background: The majority of patients with high-risk neuroblastoma (HR-NB) in low- and middle-income countries (LMIC) do not have access to autologous stem cell transplant (ASCT) and dinutuximab. Consolidation with non-myeloablative chemotherapy is not well-defined, and the outcomes are variable. We report a single-center outcome of patients with HR-NB, treated with non-myeloablative consolidation. A tabulated compilation of similar reports is included. Procedure: A retrospective chart review of patients with HR-NB was performed from January 2009 till June 2016. Patients were treated on the backbone of HR-NBL1/SIOPEN protocol. Treatment included induction with rapid-COJEC, surgery, consolidation, radiotherapy to the primary tumor, and differentiation therapy with isotretinoin. Consolidation included 4 cycles of topotecan, vincristine, and doxorubicin (TVD) instead of ASCT. Infusion of vincristine and doxorubicin were modified for ease and to enable administration in daycare. Results: Over 7-½ years, 28 patients with HR-NB were treated. Two (7%) patients had therapy-related mortality. A relapse or disease progression occurred in 11 (39%) patients at a median duration of 17 months (IQR: 5, 18). Treatment abandonment was observed in 4 (14%) patients. The 4-year event-free survival was 29.3%. The median follow up of disease-free patients is 49 months (IQR: 45, 79). Patients with relapse were not treated further. Conclusions: A 4-year EFS of 29.3% was observed when 4-cycles of TVD were administered instead of ASCT in patients with HR-NB. The study and the review will aid stakeholders in LMIC for decision-making while considering the options of treatment for HR-NB if access to ACST and dinutuximab is lacking.

Amita Trehan

and 2 more

The 2019 novel coronavirus disease (COVID-19) has affected all aspects of life globally and care of children with malignancies is no exception. We are part of a large tertiary care 2000 bedded university hospital in North India. Approximately 450 new malignancies are diagnosed annually, translating to 10 patients per week. About 200 children attend the clinics every week. Additionally, 15-20 walk-in patients are evaluated daily in the daycare. Patients travel from distances as far as 500 km to reach our center.  Merely 20% live within 50 km of the hospital, and approximately 35% reside at a distance exceeding 200 km. Two-third of the patients hail from urban areas and one third belong to rural areas. The majority of families stay in a patient hostel in the hospital premises, and a few rent a flat in the city for the 4-9 months duration of intensive phase of therapy. The Government of India mandated a lockdown on the 24th March 2020 in response to the coronavirus pandemic and the outpatient services of the hospital were closed. The borders with the neighboring states were sealed & vehicular movement curtailed with barring of public transport & suspension of all interstate and intercity travel. The citizens were advised to maintain social distancing. The unit was faced with the formidable challenge of ensuring the well-being of children under our care from a wide geographical spread with minimal access to Pediatric Oncology services in their   hometowns. How did we manage our patients?