ABSTRACT
The 2021 World Health Organization Classification of Tumors of the Central Nervous System reflected advances in understanding of the roles of oncohistones in gliomagenesis with the introduction of the H3.3-G34R/V mutant glioma to the already recognized H3-K27M altered glioma, which represent the diagnoses of pediatric-type diffuse hemispheric glioma and diffuse midline glioma, respectively. Despite advances in research regarding these disease entities, the prognosis remains poor. While many studies and clinical trials focus on H3-K27M-altered-glioma patients, those with H3.3-G34R/V mutant gliomas represent a particularly understudied population. Thus, we sought to review the current knowledge regarding the molecular mechanisms underpinning the gliomagenesis of H3.3-G34R/V mutant gliomas and the diagnosis, treatment, long-term outcomes, and possible future therapeutics.
ABSTRACT
The authors describe the first documented case of transplacental transmission of metastatic melanoma to the neuraxis. The patient was a 7-month-old boy who presented with signs and symptoms of elevated intracranial pressure. Magnetic resonance imaging revealed an inhomogeneously enhancing posterior fossa mass measuring 5 x 5 x 6 cm and filling the fourth ventricle. A posterior fossa craniotomy was performed. Pathological studies confirmed the presence of a metastatic melanoma that was pathologically identical to that of his mother. The boy received aggressive chemotherapy and underwent an additional resection. He also required a ventriculoperitoneal shunt for treatment of his hydrocephalus. He lived longer than any other patient with transplacental transmission of metastatic melanoma but ultimately died of the disease, 18 months after his initial presentation.
