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BACKGROUND: H3 K27-altered diffuse midline gliomas (DMGs) are rare tumors, which are, regardless of their histological appearance, classified as World Health Organization grade 4 tumors. They are characterized by a diffuse growth pattern, midline anatomical location, and poor prognosis. Although DMGs occur predominantly in childhood, these tumors can also be found in young adults. OBSERVATIONS: The authors present a case of a 29-year-old patient who was found unconscious with a Glasgow Coma Scale score of 4, along with abnormal extensor movements and bilateral middilated nonreactive pupils. Computed tomography revealed obstructive hydrocephalus due to an acute hemorrhage in a right thalamic lesion. To drain the hydrocephalus and relieve the ongoing central herniation, emergent placement of a right-sided, and later a left-sided, extraventricular drain was performed. Despite the postoperative resolution of hydrocephalus, the patient died shortly after because of the central brain herniation that had occurred. Brain autopsy revealed a H3 K27-altered DMG in the right thalamus. LESSONS: Although typically described in the pediatric population and located in the pons, H3 K27-altered DMG should also be considered in young adult patients with midline lesions, particularly if they are located in the thalamus or brainstem. In rare cases, H3 K27-altered DMG may present with an acute tumor-related hemorrhage, leading to a fulminant clinical course.
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Background: Tumors of the vertebral column consist of primary spinal tumors and malignancies metastasizing to the spine. Although primary spine tumors are rare, metastases to the spine have gradually increased over past decades because of aging populations and improved survival for various cancer subtypes achieved by advances in cancer therapy. Metastases to the vertebral column occur in up to 70% of cancer patients, with 10% of patients demonstrating epidural spinal cord compression. Therefore, many cancer patients may face spinal surgical intervention during their chronic illness; such interventions range from simple cement augmentation over decompression of neural elements to extended instrumentation or spinal reconstruction. However, precise surgical treatment guidelines do not exist, likely due to the lack of robust, long-term clinical outcomes data and the overall heterogeneous nature of spinal tumors. Objectives of launching the Swiss Spinal Tumor Registry (Swiss-STR) are to collect and analyze high-quality, prospective, observational data on treatment patterns, clinical outcomes, and health-related quality of life (HRQoL) in adult patients undergoing spinal tumor surgery. This narrative review discusses our rationale and process of establishing this spinal cancer registry. Methods: A REDCap-based registry was created for the standardized collection of clinical, radiographic, surgical, histological, radio-oncologial and oncological variables, as well as patient-reported outcome measures (PROMs). Discussion: We propose that the Swiss-STR will inform on the effectiveness of current practices in spinal oncology and their impact on patient outcomes. Furthermore, the registry will enable better categorization of the various clinical presentations of spinal tumors, thereby facilitating treatment recommendations, defining the socio-economic burden on the healthcare system, and improving the quality of care. In cases of rare tumors, the multi-center data pooling will fill significant data gaps to yield better understanding of these entities. Finally, our two-step approach first implements a high-quality registry with efficient electronic data capture strategies across hospital sites in Switzerland, and second follows with potential to expand internationally, thus fostering future international scientific collaboration to further push the envelope in cancer research.