Posterior reversible encephalopathy syndrome in immunocompromised children - A single-center study from South India.

This study describes the profile of children diagnosed with posterior reversible encephalopathy syndrome (PRES) in the pediatric hematology oncology unit and highlights the clinical features of PRES in immunosuppressed children. This retrospective study included 10 children diagnosed with PRES with a mean age of 6.8 years.

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder of immune dysregulation associated with significant challenges in diagnosis and management. Described as primary HLH secondary to genetic defects or more commonly secondary to infections, it can also occur secondary to malignancy, i.e.

Preoperative three-dimensional modelling and virtual reality planning aids nephron sparing surgery in a child with bilateral Wilms tumour.

Bilateral Wilms tumour (BWT) is a surgically challenging condition. Virtual reality (VR) reconstruction aids surgeons to foresee the anatomy ahead of Nephron Sparing Surgery (NSS). Three-dimensional (3D) visualisation improves the anatomical orientation of surgeons performing NSS.

Isolated Prothrombin Deficiency: A Case Report of a Rare Coagulation Disorder and Review of Literature.

Congenital prothrombin deficiency is a rare hemorrhagic disorder, frequent in areas with high degrees of consanguinity as it is autosomal recessive in nature. Clinical manifestations are highly variable, ranging from mild episodes of bleeding to severe hemorrhages. Here, we report a child with isolated prothrombin deficiency who presented with a history of pain and soreness in the prepuce associated with bleeding.

Evaluation of Pathway to Diagnosis of Pediatric Brain Tumors in Tamil Nadu, India.

Purpose: Delayed diagnosis and poor awareness are significant barriers to the early intervention of pediatric brain tumors. This multicenter observational study aimed to evaluate the baseline routes and time to diagnosis for pediatric brain tumors in Tamil Nadu (TN), with the goal of promoting early diagnosis and timely referrals in the future.

Methods: A standard proforma was used to retrospectively collect information on demographics, diagnosis, referral pathways, and symptoms of incident pediatric brain tumor cases between January 2018 and October 2020 across eight tertiary hospitals in TN.

Experience with Generic Pegylated L-asparaginase in Children with Acute Lymphoblastic Leukemia from a Tertiary Care Oncology Center in South India.

Dhaarani Jayaraman  Acute lymphoblastic leukemia (ALL) is a common type of leukemia in children. The innovator pegylated L-asparaginase has several advantages over native L-asparaginase; however, its use in India is limited due to availability and cost. Therefore, a generic pegylated L-asparaginase can be considered as an alternative to the innovator molecule.

Role of Polymerase Chain Reaction-Based Diagnosis of Respiratory Viruses in Febrile Neutropenic Patients.

Background Neutropenic patients are commonly affected by respiratory infections, whereas respiratory viral infections causing high morbidity and mortality are routinely diagnosed in developing countries like India. Our study aimed to investigate the prevalence of respiratory viral infections in pediatric cancer patients with febrile neutropenia.  Methods This prospective study was performed on 45 neutropenia patients with hematological malignancies.

Successful Outcome of Metastatic Pancreatoblastoma in an Adolescent: A Case Report and Review of the Literature.

Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers.

Renal Primitive Neuroectodermal Tumor Mimicking Wilms' Tumor in a Young Boy: A Case Report of a Rare Entity with Review of the Literature.

Primary renal primitive neuroectodermal tumors (PNET) are an extremely rare entity. The tumor is very aggressive presenting with metastasis and carries a dismal prognosis. We describe the case of renal PNET in an 11-year-old boy with a solid cystic lesion in the right kidney with a thrombus in the inferior vena cava and lung nodules, mimicking Wilms' tumor.

Disseminated Cytomegalovirus Infection in a Child with Langerhans Cell Histiocytosis.

Cytomegalovirus (CMV) reactivation is well known in post-transplant immunocompromised children. However, the incidence in non-transplant patients is significantly less, and only scarce case reports are available in the literature regarding CMV disease in children with solid tumors. We present a 3-year-old male child with multisystem refractory Langerhans cell histiocytosis, who had very high CMV viremia and disseminated CMV infection with secondary hemophagocytic lymphohistiocytosis and was successfully treated without organ damage and sequelae.

Nephrotic Syndrome and Hodgkins Lymphoma - an Unusual Association.

Background: An association between nephrotic syndrome and extrarenal neoplasia was described for the first time in 1922. The reported incidence of nephrotic syndrome in Hodgkin lymphoma is less than 1%.

Clinical Description: We present a 13 year old boy who was admitted with complaints of abdominal pain, vomiting and loose stools for 2 months.

Mercaptopurine induced myelosuppression in a child with a rs116855232 homozygous variant.

Introduction: Mercaptopurine (6-MP) is the backbone of the consolidation and maintenance therapy for paediatric acute lymphoblastic leukaemia (ALL). Nevertheless, it can cause critical myelosuppression. Predicting adverse reactions to 6-MP often involves the investigation of pharmacogenetic variants; in particular thiopurine S-methyltransferase () and nudix hydrolase 15 ().

Severe diabetic ketoacidosis and autoimmune pancreatitis with SIRS in an adolescent with LRBA deficiency - A rare complication of a common primary immunodeficiency disease.

Common variable immunodeficiency is the most common primary immunodeficiency disorder. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency is categorized as a common variable immunodeficiency associated with autoimmune manifestations and inflammatory bowel diseases. We report a rare case, an adolescent presenting with severe diabetic ketoacidosis (DKA) and acute pancreatitis with multiorgan dysfunction with common variable immunodeficiency (CVID) with homozygous mutation.

Telangiectatic Osteosarcoma in a Young Child - A Case Report and Review of the Literature.

Introduction: Osteosarcoma (OS) is one of the most common primary malignant bone tumors in children and adolescents. OS incidence varies significantly with age and peak incidence is in adolescent age group. Telangiectatic osteosarcoma (TOS) is an unusual variant of OS, forming 3-10% of all OSs.

CARMIL2 Immunodeficiency with Epstein Barr Virus Associated Smooth Muscle Tumor (EBV-SMT). Report of a Case with Comprehensive Review of Literature.

Primary immunodeficiency (PID) having defects related to lymphocyte cytotoxic pathway or T-cell dysfunction are well known for developing opportunistic infections and Epstein-Barr virus (EBV)-associated diseases. CARMIL2 deficiency is a recently described combined immunodeficiency (CID) disorder characterized by defective CD28-mediated T cell co-stimulation, altered cytoskeletal dynamics, susceptibility to various infections and Epstein Barr Virus smooth muscle tumor (EBV-SMT). We report a homozygous CARMIL2 pathogenic variant presenting with recurrent infections and EBV associated smooth muscle tumor (SMT) in a child.

A Journey Across Oceans With a Heavy Heart: Rare Presentation of a Pediatric Malignancy.

Myeloid sarcoma, due to extramedullary deposition of myeloblasts, is one of the rare presentations in acute myeloid leukemia. We present an extremely rare case of a 5-year-old boy with cardiac myeloid sarcoma. Noninvasive mode of diagnosis, timely initiation of chemotherapy and meticulous supportive care are the keys to successful outcome.

Mesenchymal Hamartoma of the Chest Wall in an Infant - A Benign Entity Masquerading as Malignancy.

Mesenchymal hamartoma of the chest wall is a rare tumor-like lesion encountered in neonates and infants. Although typically benign with no metastatic potential, it has alarming imaging and pathological features that mimic malignancy. We describe the imaging, surgical, and pathological features of this rare entity in a 1-month-old infant.

Oropharyngeal Kimura's disease: a diagnostic dilemma and therapeutic challenge.

Kimura's disease is a chronic inflammatory disorder involving subcutaneous tissue and locoregional lymph nodes of head and neck region, characterised by angiolymphoid proliferation. It is typically associated with peripheral eosinophilia, increased serum IgE and lymphadenopathy. Diagnosis is usually based on histopathological examination.