A 17-year-old girl with World Health Organization grade IV glioblastoma with primitive neuronal components (histone H3 G34-mutant and IDH1 wild type) underwent whole-body FDG PET/CT staging due to vertebral metastases on initial MRI. PET/CT revealed extracranial metastatic disease with spinal leptomeningeal dissemination, osseous metastases, and peritoneal seeding via a ventriculoperitoneal shunt. Glioblastoma is uncommon in pediatric patients and particularly those with primitive neuronal components. Extracranial metastases from glioblastoma are more common in those with primitive neuronal components. This case demonstrates the utility of FDG PET/CT for revealing distant metastases from glioblastoma.
Ovarian angiosarcoma is a rare and aggressive vascular tumor, which has a 5‐year overall survival of less than 30% for patients with nonmetastatic disease and almost certain death within 1 year for those with metastasis. Here, we briefly review historical approaches to therapy and present a long‐term survivor in the case of an 11‐year‐old female with metastatic ovarian angiosarcoma. This is the second reported case to utilize heated intraperitoneal chemotherapy in the treatment of this disease. Our patient is currently alive and well 3 years after initial diagnosis, significantly longer than any reported case of advanced‐stage ovarian angiosarcoma.
Hypodiploid acute lymphoblastic leukemia (ALL) is an aggressive blood cancer with a poor prognosis despite intensive chemotherapy or stem cell transplant. Children and adolescents with positive end-of-induction minimal residual disease (MRD) have an overall survival lower than 30%. However, data regarding therapeutic alternatives for this disease is nearly nonexistent, emphasizing the critical need for new or adjunctive therapies that can improve outcomes. We previously reported on the therapeutic efficacy of venetoclax (ABT-199) in hypodiploid B-ALL but with limitations as monotherapy. In this study, we set out to identify drugs enhancing the anti-leukemic effect of venetoclax in hypodiploid ALL. Using a high throughput drug screen, we identified dinaciclib, a cyclin-dependent kinase (CDK) inhibitor that worked synergistically with venetoclax to induce cell death in hypodiploid cell lines. This combination eradicated leukemic blasts within hypodiploid ALL PDX mice with low off-target toxicity. Our findings suggest that dual inhibition of BCL-2 (venetoclax) and CDK9/MCL-1 (dinaciclib) is a promising therapeutic approach in hypodiploid ALL, warranting further investigation to inform clinical trials in this high-risk patient population.
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