Protocol for ICiCLe-ALL-14 (InPOG-ALL-15-01): a prospective, risk stratified, randomised, multicentre, open label, controlled therapeutic trial for newly diagnosed childhood acute lymphoblastic leukaemia in India.

Background: In the west, survival following treatment of childhood acute lymphoblastic leukaemia (ALL) approaches 90%. Outcomes in India do not exceed 70%. To address this disparity, the Indian Collaborative Childhood Leukaemia group (ICiCLe) developed in 2013 a contemporary treatment protocol for uniform risk-stratified management of first presentation ALL based on cytogenetics and minimal residual disease levels (MRD).

The Spectrum of Clinical, Immunological, and Molecular Findings in Familial Hemophagocytic Lymphohistiocytosis: Experience From India.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune dysregulation characterized by hyperactivation of the immune system, excessive cytokine secretion and severe systemic inflammation. HLH is classified as familial (FHL) when associated with mutations in , and genes. There is limited information available about the clinical and mutational spectrum of FHL patients in Indian population.

Unfavorable presentation but comparable outcome: Presentation and outcome of children with nodular lymphocyte predominant Hodgkin lymphoma from India.

Background: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma (HL) with few published studies in children, entirely from North America and Europe. We analyzed clinical features and treatment outcome of pediatric NLPHL.

Procedure: Children less than 18 years of age diagnosed after histopathology review to have NLPHL between June 1998 and August 2016 were retrospectively analyzed.

Applicability of 2008 World Health Organization classification system of hematolymphoid neoplasms: Learning experiences.

Background: 2008 World Health Organization (WHO) classification of hematolymphoid neoplasms (HLN) has classified them based on morphology, results of various ancillary techniques, and clinical features. There are no studies looking at the applicability of WHO classification.

Aims: The aim of the study was to calculate proportions of all HLN subtypes seen during 1-year period based on 2008 WHO classification of HLN and study applicability and also shortcomings of practices in a tertiary care center in India.

Treatment of Langerhans cell histiocytosis with a modified risk-adapted protocol-experience from a tertiary cancer institute in India.

Background: Involvement of risk-organs (RO+) in Langerhans cell histiocytosis (LCH) and inadequate early response identifies patients at high risk for relapse and mortality requiring intensive salvage therapy including stem cell transplant, adding cost and toxicity. To mitigate this, we used a standard induction, augmented with metronomic etoposide, and prolonged maintenance-similarly augmented for RO+, and retrospectively analyzed its impact.

Procedure: LCH patients from 2009 through 2014 were included.

Story of survival in anaplastic large cell lymphoma - sometimes more than the anaplastic lymphoma kinase status: An evaluation of pathologic prognostic factors in 102 cases.

Introduction: Systemic anaplastic large cell lymphoma (ALCL) accounts for 5%-10% of adult non Hodgkin's lymphoma (NHL) and 10%-30% of childhood NHL. Owing to significant differences in survival and gene expression profile, current WHO classifies ALCL into two distinct entities as anaplastic lymphoma receptor tyrosine kinase (ALK) positive and ALK negative ALCL with ALK expression by tumour as a good prognostic indicator. However, in our institute which is a cancer referral institute, our preliminary experience was that even ALK positive tumours did not fare well as compared to ALK- negative ALCL.

A Rare Extramedullary and Extralymphoid Presentation of Mixed Phenotypic Blastic Hematolymphoid Neoplasm: A Study of Two Cases.

Mixed phenotype acute leukemia (MPAL) is a rare hematolymphoid neoplasm, representing only 3%-5% of acute leukemia. Although MPAL has been sufficiently described in the literature, its extramedullary presentation as a solitary lesion without leukemic (bone marrow [BM]) involvement is rarely described. We are presenting two cases of mixed phenotypic blastic hematolymphoid neoplasms without leukemic involvement at disease presentation in 8-year-old female and 21-year-old male patients.

Traditional and Complementary Medicine in Pediatric Oncology and Low-Middle Income Countries: Recommendations from the International Society of Pediatric Oncology (SIOP), T&CM Collaborative.

Significant strides have been made in the treatment of childhood cancer. Improvements in survival have led to increased attention toward supportive care indications; including the use of traditional and complementary medicine (T&CM). The use of T&CM among children and adolescents with cancer is well documented in both high-income countries (HICs) and low-middle income countries (LMICs).

Pain Management and Use of Opioids in Pediatric Oncology in India: A Qualitative Approach.

Purpose: Consumption of medical opium for pain relief in India is low, despite the country being one of the main world producers of the substance. We investigated obstacles to opioid use and physician perceptions about optimal pain management in pediatric oncology patients in India.

Methods: Semistructured interviews were conducted with oncologists who work in pediatric oncology settings.

An integrated genomic profile that includes copy number alterations is highly predictive of minimal residual disease status in childhood precursor B-lineage acute lymphoblastic leukemia.

Introduction: Copy number alterations (CNA) have been described in childhood precursor B-lineage acute lymphoblastic leukemia (B-ALL) which in conjunction with chromosomal abnormalities drive leukemogenesis. There is no consensus on the clinical incorporation of CNA in B-ALL. An integrated genomic classification (IGC) has been proposed which includes CNA and cytogenetics.

Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document.

Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children.

Management of Non-Hodgkin Lymphoma: ICMR Consensus Document.

Hitherto poor outcomes, paucity of data and heterogeneity in International approach to Pediatric NHL (Non-Hodgkin Lymphoma) prompted the need for guidelines for Indian population with vast variability in access, affordability and infrastructure across the country. These guidelines are based on consensus among the experts and best available evidence applicable to Indian setting. Evaluation of NHL should consist of easily doable and rapid tissue diagnosis (biopsy or flow cytometry of peripheral blood/malignant effusions), St Jude/IPNHLSS (International Pediatric Non-Hodgkin Lymphoma Staging System) and risk grouping with CSF (Cerebro-spinal fluid), bone marrow, whole body imaging [CECT (Contrast enhanced computerized tomography) ± MRI (Magnetic resonance imaging)] and blood investigations for LDH (Lactate dehydrogenase), TLS (Tumor lysis syndrome) and organ functions.

Management of Neuroblastoma: ICMR Consensus Document.

Neuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain.

Management of Wilms Tumor: ICMR Consensus Document.

Wilms tumor (WT) is the most common renal tumor of childhood. Although multidisciplinary care including surgery, chemotherapy and radiotherapy have greatly improved the survival rates in WT, there is a scope for further improvement in India and other resource-poor settings. In resource-limited settings, the majority of patients present with large tumors, which may either be unresectable or risky to resect; making preoperative chemotherapy followed by delayed surgery the preferred approach.

Management of Pediatric Malignant Germ Cell Tumors: ICMR Consensus Document.

With the introduction of cisplatin, the outcome of children with malignant germ cell tumors (MGCT) has improved to nearly 90% 5 year survival. Over the years, through the results of various multinational co-operative trials, the chemotherapy and surgical guidelines for both the gonadal and extra-gonadal MGCTs have been refined to decrease the early and late morbidities and at the same time improve survival. Introduction of risk categorization has further added to this effort.

Management of Hodgkins Lymphoma: ICMR Consensus Document.

Pediatric Hodgkins lymphoma is a highly curable disease even in the developing world. Current treatment paradigms follow a risk and response based approach. The goal is to minimise treatment related short and long-term toxicity while maintaining excellent survival.

Management of Hepatoblastoma: ICMR Consensus Document.

Dramatic advancement has been made in the management of children with hepatoblastoma (HB) over the past 3 decades owing to the improvement in diagnostic imaging, new chemotherapeutic agents, better surgical care and availability of liver transplantation. These advances are the end results of contributions from 4 major study groups across the globe including International Society of Pediatric Oncology - Liver Tumor Strategy Group (SIOPEL), Children's Oncology Group (COG), German Pediatric Hematology Oncology Group (GPOH) and Japanese Pediatric Liver Tumor Study Group (JPLT). The current manuscript is written with the objective of developing a consensus guideline for practitioners at a National level.

Intracranial germ cell tumors: a multi-institutional experience from three tertiary care centers in India.

Objective: Central nervous system germ cell tumors (CNS GCTs) are relatively rare neoplasms. Incidence of CNS GCTs in Western literature is low (0.3-0.

Evaluation of new markers for minimal residual disease monitoring in B-cell precursor acute lymphoblastic leukemia: CD73 and CD86 are the most relevant new markers to increase the efficacy of MRD 2016; 00B: 000-000.

Background: Multiparametric flow cytometry (MFC) is a popular technique for minimal residual disease (MRD) analysis. However, its applicability is still limited to 90% of B-cell precursor acute lymphoblastic leukemia (BCPALL) due to two major issues, i.e.

Acute leukemia in children: A review of the current Indian data.

Acute leukemias are the most common diagnostic group of childhood cancer. This review summarizes the published literature on reported current outcomes of childhood acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) from India. Overall survival in ALL ranged from 45% to 81% (commonly >60%) and event-free survival ranged from 41% to 70% (commonly >50%).

'Childhood systemic mastocytosis associated with t (8; 21) (q22; q22) acute myeloid leukemia'.

Systemic mastocytosis (SM) with associated clonal nonmast cell lineage disease is seen in up to 20% cases of SM. SM is uncommon in the pediatric population. T (8; 21) (q22; q22) is a good prognostic factor in acute myeloid leukemia (AML).

A Framework for Adapted Nutritional Therapy for Children With Cancer in Low- and Middle-Income Countries: A Report From the SIOP PODC Nutrition Working Group.

The utilization of adapted regimens for the treatment of pediatric malignancies has greatly improved clinical outcomes for children receiving treatment in low- and middle-income countries (LMIC). Nutritional depletion has been associated with poorer outcomes, increased abandonment of therapy, and treatment-related toxicities. Surveys have found that nutritional intervention is not incorporated routinely into supportive care regimens.

Characteristics of BCR-ABL kinase domain mutations in chronic myeloid leukemia from India: not just missense mutations but insertions and deletions are also associated with TKI resistance.

We document the characteristics of BCR-ABL kinase domain mutations (KDM) in the largest study from India comprising of 385 patients and demonstrate that more than half (51.9%) of these patients have detectable abnormalities in the KD both in adult and in pediatric chronic myelogenous leukemia (CML). These comprise singly occurring missense mutations (25.