An Immersive Virtual Reality Curriculum for Pediatric Hematology Clinicians on Shared Decision-making for Hydroxyurea in Sickle Cell Anemia
Although hydroxyurea (HU) is an effective treatment for sickle cell anemia, uptake remains low. Shared decision-making (SDM) is a recommended strategy for HU initiation to elicit family preferences; however, clinicians lack SDM training. We implemented an immersive virtual reality (VR) curriculum at 8 pediatric institutions to train clinicians on SDM that included counseling virtual patients. Clinicians’ self-reported confidence significantly improved following the VR simulations on all communication skills assessed, including asking open-ended questions, eliciting specific concerns, and confirming understanding (Ps≤0....
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Regression in Early Childhood Development: An Unintended Outcome of Prolonged Hospitalization?
This brief review outlines a novel case study with targeted literature search. Patient X was a 21-month-old male who was receiving ongoing treatment for stage M MYCN-amplified high-risk neuroblastoma. Patient X’s mother was considering refusal of further cancer-directed therapy because of the child’s developmental regression noted during his prolonged hospitalization. Given the underlying malleability of the developing brain in early childhood, access to supportive services that facilitate ongoing neurodevelopment in hospitalized young children is of utmost importance; such services further reduce parental stress and l...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Suspected Drug-induced Liver Injury Due to 6-Mercaptopurine With a Superimposed SARS-CoV-2 Infection in a Patient With B-ALL
This report describes a case of suspected drug-induced liver injury exacerbated by a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A 16-year-old male with very high risk B-cell acute lymphoblastic leukemia was admitted for hyperbilirubinemia 2 months after a 6-mercaptopurine dosage increase and found to have an active SARS-CoV-2 infection. Liver function improved throughout hospitalization and the patient was discharged on allopurinol. Following liver function after a dosage increase of hepatoxic chemotherapy and in a pediatric oncology patient with an active SARS-CoV-2 infection undergoing treatm...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Treatment of Sinusoidal Obstruction Syndrome With Defibrotide in Pediatric Cancer Patients Following Nontransplant-associated Chemotherapy: A Case Report and Review of the Literature
We report a 3-year-old with relapsed Wilms tumor and recurrent SOS/VOD, with successful use of defibrotide during chemotherapy. A review of pediatric cancer patients with nontransplant-associated SOS/VOD treated with defibrotide revealed 83 patients, and 66 were in remission. This review supports early treatment with defibrotide in patients with nontransplant-associated SOS/VOD. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Bone Health in Osteosarcoma at Presentation and Its Impact on Cancer Treatment: A Case Series of 3 Pediatric Patients
Osteosarcoma is the most common pediatric malignant bone tumor. Concomitant osteoporosis has typically been attributed to oncologic therapy. The present case series is aimed to describe 3 patients who presented with osteoporosis or osteopenia before, or early in, their oncology treatment. In our patients, bone health and its complications had significant impacts including pain, reduced mobility, prolonged admission, and delays in recovery. Our patients experienced improvement with resection of their primary tumor and with bisphosphonate infusion. Future studies are required to determine the prevalence osteoporosis at prese...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Infantile Stage M Neuroblastoma With 11q Deletion, Mimicking Stage MS
A 4-month-old boy with abdominal distension was diagnosed with adrenal neuroblastoma with numerous metastases to the liver and nodules in the skin and muscles. Marked hepatomegaly spontaneously regressed with decreasing tumor marker levels, and the final diagnosis was stage M based on radiologic findings confirming metastasis to the pancreas. The neuroblastoma did not have the MYCN amplification but had an 11q aberration. Chemotherapy was initiated at age 6 months with a successful response. Our case reflects the heterogenous clinical behavior of neuroblastoma and highlights the challenging issue of the difference between ...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Two Histologic Patterns of Lymphomatoid Papulosis Occurring in a Child: A Matter of Timing?
We report a case of lymphomatoid papulosis presenting with 2 histologic subtypes (types A and B) occurring concomitantly in a 10-year-old child, and postulate that the different subtypes occur dependent on the age of the lesion biopsied. Incidentally, one of the biopsies also shows a rarely seen pattern of pseudoepitheliomatous hyperplasia in a pediatric lymphomatoid papulosis patient. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Undifferentiated and Unresectable Sarcoma With NTRK3-Fusion in a Pediatric Patient Treated With Larotrectinib and Proton Beam Radiotherapy
A 6-year-old female presenting with an abdominal mass was found to have an unresectable undifferentiated sarcoma. The tumor did not respond to multiagent chemotherapy. However, molecular testing identified an NTRK3-fusion, and treatment was changed to larotrectinib monotherapy. Following 6 months of therapy, the patient achieved a very good partial response with 96% reduction in tumor size. She underwent proton beam radiation therapy with continued larotrectinib therapy and achieved a complete response. This case report shows that an NTRK fusion positive undifferentiated sarcoma can be safely treated with larotrectinib and...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hemiplegic-Migraine–like Attacks as First Manifestation of Diffuse Leptomeningeal Glioneuronal Tumor: A Case Report
Conclusions: This is the first report of HM-like attacks in DLGNT. We discuss the pathogenetic hypotheses of our case and previously reported cases of “symptomatic” HM with leptomeningeal involvement. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Clinicopathologic Profile and Treatment Outcomes of Children With Diffuse Large B-cell Lymphomas: Experience From a Tertiary Cancer Center in India
Clinicopathologic profile and outcome of 15 children (15 years or above) with diffuse large B-cell lymphoma treated with MCP-842 protocol are reported. Eleven of 15 presented with advanced (stage-III/IV) disease. Post-2 cycles of chemotherapy, complete metabolic and morphologic response was documented in 10 (66%) and rest 5 (33%) with partial response achieved complete metabolic remission by end of treatment. At a median follow-up of 44 months (range: 16 to 79 mo), the 3-year event-free survival and overall-survival were 77.1%±11.7% and 85.7%±9.4%, respectively. Though majority of our patients had advanced disease, out...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

T-cell Lymphoblastic Lymphoma in a Patient With Chromosome 8q21.11 Microdeletion
We report a case of a 2-year-old female with chromosome 8q21.11-q21.2 microdeletion complicated by T-cell lymphoblastic lymphoma. Whole genome single-nucleotide polymorphism microarray detected an interstitial deletion of 8q21.11 to q.21.2, including 16 genes. Autopsy findings revealed a T-cell lymphoblastic lymphoma presenting as an anterior mediastinal mass, encroaching upon the aortic arch, left subclavian artery, left carotid bifurcation and trachea. The genes that may contribute to a neoplastic process are identified (PKIA, IL7, TPD52, PAG1, and FABP5) and discussed in this article. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Angiosarcoma of the Pancreas in a Pediatric Patient With an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature
Pancreatic angiosarcoma is an exceedingly rare malignancy accounting for (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Juvenile Xanthogranuloma of the Pancreas in a Pediatric Patient Mimicking Pancreatic Neoplasm With High CA 19-9: Case Report and Literature Review
We present an unusual case of a 13-month-old female child with JXG of the pancreas and elevated cancer antigen 19-9. JXG should always be considered as a differential diagnosis for pediatric patients presenting with a pancreatic mass, solid and/or cystic in nature. Therefore, avoiding unnecessary invasive diagnostic procedures. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

An Atypical Case of Very Early-onset Familial Adenomatous Polyposis Associated With Focal Cortical Dysplasia
We describe a female toddler with rectal bleeding from extensive colonic polyposis, and diagnosed with familial adenomatous polyposis. She has epilepsy from infancy attributed to focal cortical dysplasia. Hepatoblastoma was diagnosed at 13 months of age. Germline testing detected a pathogenic APC (adenomatous polyposis coli gene) variant. We discuss the anecdotal management of this case, including the clinical utility of genetic confirmation. We review the genotype-phenotype correlation of the APC mutational spectrum, and the existing evidence supporting the hypothesis that cortical dysplasia is part of the APC-related spe...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern
We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research