Browsing by Author "Tailor, Jignesh"

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    Human stem cell models to unravel brain cancer
    (Springer Nature, 2024-11-28) Dave, Biren; Tailor, Jignesh; Neurological Surgery, School of Medicine
    Pre-clinical animal models of human brain tumors have been invaluable tools for studying cancer pathogenesis and exploring novel treatment modalities. Such models recapitulate important aspects of the human disease such as the stem-progenitor-differentiated cell hierarchy. Although powerful, we argue that animal models are inherently limited in their ability to phenocopy certain important aspects of human brain tumor biology. We specifically highlight the inability of mouse models to generate certain forms aggressive pediatric medulloblastoma likely owing to cellular, anatomic, and genetic differences between the human and mouse brains. Additionally, we review some limitations of human brain tumor derived cell lines and outline why they are a sub-optimal system for purposes of pre-clinical modeling. Below, we present the case for human stem cell-based models of brain tumors, focusing mainly on glioblastoma and medulloblastoma. Drawing on several recently published studies, we review the exciting progress that has been made towards modeling human brain tumors using two-dimensional adherent stem cell cultures and three-dimensional organoids. We identify the important advances arrived at using these human stem cell-based models and suggest opportunities for future work in this direction. In this review article, we aim to highlight the utility and promises of human stem cell-based models of brain tumors as a complementary system to traditional transgenic animal and cell line systems.
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    NCOG-25. Single-Institution Series of Spinal Ependymoma in Children With NF2-Related Schwannomatosis
    (Oxford University Press, 2023-11-10) Burket, Noah; Koenig, Jenna; Tailor, Jignesh; Neurological Surgery, School of Medicine
    NF2-related schwannomatosis (NrS) is a tumor predisposition syndrome that results in the development of multiple central nervous system tumors, including spinal ependymomas (SP-EPN). SP-EPN are intramedullary tumors that are slow growing but can displace critical nerve pathways in the spinal cord, resulting in motor and sensory deficits that contribute to patient morbidity. Although surgery is the mainstay of treatment, it can also result in high morbidity, resection of sporadic SP-EPN is often curative; however, SP-EPN in NrS patients will often regrow, possibly resulting in additional surgeries and a lifetime of deficits, especially impacting pediatric patients. Yet, natural history and clinical outcomes for pediatric NrS patients with SP-EPN have not been well described. We reviewed 27 pediatric NrS patient cases from 1993 to 2023 at our institution and identified 10 patients with a diagnosis of SP-EPN, with a range of 1-3 tumors present at patient diagnosis. Demographic, clinical, pathologic, and radiologic data were collected from electronic medical records. Median age of diagnosis of NrS was 12 years old (range: 5-17) and median time from NrS diagnosis to SP-EPN diagnosis was 31 days (range: -1-1636). 14 out of 16 (87.5%) initial tumors were in the cervical spine region, with C2 being the most frequently involved level. Two patients underwent resection of their tumors, with both experiencing progression of their disease and one developing paraplegia. The most common symptoms from SP-EPN were pain (30%), motor deficits (30%), and sensory deficits (30%). 40% of the patients had progressive disease at last follow up, with median time from diagnosis to progression of 3.33 years. Median time from SP-EPN to last follow-up was 4.08 years. This series showcases the burden of SP-EPN in pediatric NrS patients, emphasizing the need for further exploration into clinical outcomes and novel treatments for this debilitating condition.
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    Pediatric Prostatic Alveolar Rhabdomyosarcoma Presenting with Metastatic Spinal Cord Compression in the Thoracic Spine: A Case Report and Review of the Literature
    (Springer Nature, 2024-03-20) Perry, Matthew T.; Witten, Andrew J.; Marwan, Majeed; Vortmeyer, Alexander; Tailor, Jignesh; Neurological Surgery, School of Medicine
    Rhabdomyosarcoma (RMS) is a pediatric malignancy with a variable prognosis depending on tumor stage and genotype. There has been a significant improvement in survival rates over the past decades. However, aggressive variants can metastasize to locations that are difficult to treat. We report a case of prostatic alveolar rhabdomyosarcoma with metastases to the bone marrow and thoracic spine in a child. The patient was treated with a multimodal approach that included surgical resection of the epidural mass; the administration of vincristine, dactinomycin, and cyclophosphamide; and radiotherapy. Unfortunately, after six months, the patient showed disease progression and was started on secondary-line treatment. This case illustrates the difficulties in managing end-stage metastatic rhabdomyosarcoma and is the first report of prostatic rhabdomyosarcoma presenting with spinal cord compression in a child.