A5052825824- Glioma Diagnosis and Treatment
- Testicular diseases and treatments
- Neuroblastoma Research and Treatments
- Chromatin Remodeling and Cancer
- Cancer, Hypoxia, and Metabolism
- Proteoglycans and glycosaminoglycans research
- Hedgehog Signaling Pathway Studies
- Glycosylation and Glycoproteins Research
- Teratomas and Epidermoid Cysts
- Lung Cancer Research Studies
- Head and Neck Surgical Oncology
- Cancer Genomics and Diagnostics
- Sexual Differentiation and Disorders
- Meningioma and schwannoma management
- Hippo pathway signaling and YAP/TAZ
- Congenital Diaphragmatic Hernia Studies
- Pancreatic and Hepatic Oncology Research
- Ferroptosis and cancer prognosis
- Ocular Oncology and Treatments
- Mesenchymal stem cell research
- Neonatal Respiratory Health Research
- Management of metastatic bone disease
- Advanced Electron Microscopy Techniques and Applications
- Fibroblast Growth Factor Research
- Brain Metastases and Treatment
Children's Hospital of Los Angeles
2021-2025
University of Southern California
2022-2025
British Columbia Children's Hospital
2022-2024
APLA Health
2022
Children's Center
2022
Alberta Children's Hospital
2018-2020
University of Calgary
2008-2011
The prognosis for patients with pediatric high-grade glioma (pHGG) is poor despite aggressive multimodal therapy. Objective responses to targeted therapy BRAF inhibitors have been reported in some recurrent BRAF-mutant pHGG but are rarely sustained.
Ependymoma is a tumor of the brain or spinal cord. The two most common and aggressive molecular groups ependymoma are supratentorial ZFTA-fusion associated posterior fossa group A. In both groups, tumors occur mainly in young children frequently recur after treatment. Although mechanisms underlying these diseases have recently been uncovered, they remain difficult to target innovative therapeutic approaches urgently needed. Here, we use genome-wide chromosome conformation capture (Hi-C),...
Sonic hedgehog (SHH) medulloblastoma is the most common molecular group of infant and early childhood (iMB) has no standard care at relapse. This work aimed to evaluate post-relapse survival (PRS) explore prognostic factors patients with nodular desmoplastic (ND) and/or SHH iMB. international retrospective study included 147 subjects diagnosed relapsed Nodular Desmoplastic/SHH iMB between 1995 2017, < 6 years old original diagnosis, treated without initial craniospinal irradiation (CSI)....
Infant and young childhood medulloblastoma (iMB) is usually treated without craniospinal irradiation (CSI) to avoid neurocognitive late effects. Unfortunately, many children relapse. The purpose of this study was assess salvage strategies prognostic features patients with iMB who relapse after CSI-sparing therapy.
Abstract In Xenopus laevis embryos, heparanase, the enzyme that degrades heparan sulfate, is synthesized as a preproheparanase (XHpaL) and processed to become enzymatically active (XHpa active). A short nonenzymatic heparanase splice variant (XHpaS) also expressed. Using immunohistochemistry, Western blot, promoter analysis, we studied dynamic developmental expression of three heparanases. Our results indicate (1) all isoforms are maternally expressed; (2) XHpaS variant; (3) in early embryo,...
Heparanase is an endoglycosidase that cleaves heparan sulfate (HS) side chains from proteoglycans (HSPGs) present in extracellular matrix and cell membranes. Although HSPGs have many functions during development, little known of the role enzyme degrades HS, heparanase. We cloned characterized expression two heparanase splicing variants Xenopus laevis studied their function early embryonic development. The gene (termed xHpa) spans over 15 kb consists at least 12 exons. long (XHpaL) cDNA...
DICER1 syndrome is a tumor predisposition caused by abnormal micro-RNA processing which leads to variety of benign and malignant neoplasms in many organ systems, including the central nervous system. This paper reports case primary intracranial sarcoma, DICER1-mutant, patient with germline variant thought most likely be de novo. The ten-year-old boy who presented acutely altered level consciousness, emesis, left-sided weakness. Imaging revealed large right frontal hemorrhagic lesion, was...
Abstract BACKGROUND Primary intracranial germ cell tumors (GCTs) are rare heterogeneous tumors; germinoma accounts for two-thirds of cases. While neoadjuvant chemotherapy followed by response-based reduced radiotherapy dose and field is the established approach managing localized CNS germinomas, controversy persists regarding treatment primary metastatic disease. Additionally, limited research exists on utilization in germinoma. METHODS A retrospective multi-institutional analysis was...
Abstract BACKGROUND PDLM is a rare and aggressive disease with limited cases reported worldwide few in the pediatric population. Herein we report on clinical, histologic, molecular/genetic findings of young child rapid clinical progression. CASE STUDY A 12-year-old right-handed male presented two months intermittent fevers, progressive frontal headaches, lethargy, vomiting, weight loss, one month focal seizures altered awareness, speech arrest, eye deviation, tonic posturing. Examination...
Abstract BACKGROUND: The prognosis for pediatric high-grade glioma (pHGG) is poor despite aggressive multi-modal therapy. Objective responses to targeted therapy with BRAF inhibitors have been reported in some patients recurrent BRAF-mutant pHGG but are rarely sustained. METHODS: We performed a retrospective, multi-institutional review of treated off-label +/- MEK as part their initial RESULTS: Nineteen were identified, median age 10.7 years (range: 1.8–20.3). Histologic diagnoses included...
Abstract BACKGROUND: Embryonal tumors with multi-layered rosettes (ETMRs) are rare pediatric brain poorly defined prognostic features, standard of care treatments or outcome data. Recent data suggest that high-dose chemotherapy and radiotherapy is correlated improved survival compared to alone. CASE DESCRIPTIONS: Four patients newly diagnosed ETMRs were treated 2 cycles induction per PBTC-026 using isotretinoin, vorinostat, vincristine, cisplatin, etoposide, cyclophosphamide added...
IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization markers. We sought to evaluate the frequency, clinical characteristics and outcome in patients 21 North-American Australian institutions. Patients with diagnosed from 2000-2017 were retrospectively evaluated. Out 739 GCT diagnoses, was identified 33 (4.5%). occurred 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs), 3/472 (0.6%)...
BACKGROUND: IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization markers. We sought to evaluate the frequency, clinical characteristics and outcome in patients 21 North-American Australian institutions. METHODS: Patients with diagnosed from 2000-2017 were retrospectively evaluated. RESULTS: Out 739 GCT diagnoses, was identified 33 (4.5%). occurred 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs),...
BACKGROUND: The use of high-dose chemotherapy (HDC) and autologous hematopoietic stem cell transplant (HSCT) has been used in certain pediatric patients with brain tumours to delay/spare radiotherapy. We aimed study factors predicting a successful collection (SCC) correlate dose infused HSCT outcomes. METHODS: A retrospective chart review was undertaken for treated at our centre HDC/HSCT between 2004-2016. RESULTS: Fifty-five were identified (32 male) median age 6.3 years time SCC (range...
Abstract Purpose Primary germ cell tumors (GCTs) are the most common central nervous system (CNS) neoplasm in patients with Down syndrome (DS). However, a standard-of-care has not been established due to paucity of data. Methods A retrospective multi-institutional analysis was conducted, addition comprehensive review literature. Results Ten from six institutions (five USA, one Brazil) were identified, 31 literature 1975 2021. Of 41 total (mean age 9.9 years; 61% male), 16 (39%) had...
Purpose: Primary germ cell tumors (GCTs) are the most common central nervous system (CNS) neoplasm in patients with Down syndrome (DS). However, a standard-of-care has not been established due to paucity of data. Methods: A retrospective multi-institutional analysis was conducted, addition comprehensive review literature. Results: Ten from six institutions (five USA, one Brazil) were identified, 31 literature 1975 2021. Of 41 total (mean age 9.9 years; 61% male), 16 (39%) had...
Abstract BACKGROUND: A standard-of-care has not been established for the management of patients with Down syndrome (DS) who develop primary central nervous system (CNS) germ cell tumors (GCTs) – most common CNS neoplasm in DS despite being more susceptible to treatment-related adverse events. METHODS: Data from large academic institutions were collected and a comprehensive review medical literature was conducted. RESULTS: Ten six (five USA, one Brazil) reviewed. Additionally, thirty-one...
Abstract BACKGROUND/RATIONAL: Following initial irradiation sparing therapy, many young children with relapsed medulloblastoma can be salvaged craniospinal (CSI). However, the interval to relapse is short and neurocognitive sequelae remain a major concern. The contribution of molecular subgrouping may help refine indications modalities salvage strategies in this population. METHOD: From cohort 151 molecularly characterized medulloblastoma, subset analysis SHH was conducted describe practice...
Abstract Introduction Sustained responses to molecular targeted therapy with BRAF or without MEK inhibitors have been reported in patients recurrent BRAFV600E-mutant pediatric high-grade gliomas (pHGG). The role of upfront this population, however, has not yet established. Methods We performed a retrospective, multi-institutional record review pHGGs, treated off-label and/or as part their initial adjuvant therapy. Results Seventeen were identified (median age at diagnosis, 8.8 years, range...
Abstract A 12 year-old male presented with a 2-month history of intermittent headaches, nausea, and vomiting. Magnetic resonance imaging (MRI) the brain revealed 2.2 x 3.5 2.6 cm lobulated, sellar/suprasellar mass, mildly T1/T2 hyperintense, mild homogeneous enhancement diffusion restriction. He underwent transsphenoidal right craniotomies for gross total resection mass. Pathology demonstrated hypercellular neoplasm spindled to ovoid tumor cells arranged in fascicles tight whirls, consistent...