World Neurosurg
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The revised World Health Organization classification of central nervous system tumors, published in 2016, has recognized the H3 K27M mutation as a critical genetic signature defining a new group of infiltrative astrocytomas designated as diffuse midline glioma, H3 K27M mutant. Although most H3 K27M mutations arise in the setting of diffusely infiltrative tumors, there are rare reports of compact tumors with low-grade histologic features harboring this mutation. The prevalence and clinical significance of this mutation in pilocytic astrocytomas remain unclear. ⋯ Stereotactic biopsies may undergrade some adult thalamic pilocytic astrocytomas. Therefore, we recommend that all these tumors be evaluated for the H3 K27M mutation. Further, we think H3 K27M-mutant thalamic pilocytic astrocytomas require aggressive multimodality treatment and these treatments should be guided by the molecular findings, as opposed to the histologic ones.
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Spinal ependymomas are rare, with an incidence of 1 per 100,000. Given the paucity of data for higher grade II and III disease, the management and patterns of care require further investigation. ⋯ Approximately 40% of patients with grade III ependymomas do not receive immediate adjuvant therapy, which may be related to distance from a facility. Patients with this rare tumor may benefit from multidisciplinary care at facilities with a larger volume.
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Even though surgical conditions account for as much as 32% of the global burden of disease, 5 billion people worldwide do not have access to timely, affordable surgical and anesthetic care. Access to surgical care is separated along socioeconomic divides, and these disparities are most pronounced in low-and middle-income countries, such as the Caribbean nation of Haiti, where the availability of specialty surgical services like neurosurgery are scant, or completely absent. ⋯ The paper concludes with recommendations to guide the international neurosurgery community in future contributions.
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Primary leptomeningeal melanocytic tumors of the central nervous system are rare and, especially in the spine, less frequent compared with other entities. There is no consensus regarding the best care of these tumors. ⋯ Primary melanocytic tumors of the spine are exceedingly rare. Before surgery it is difficult to make a correct diagnosis. Usually an unexpected intraoperative finding with consecutive histopathologic analyses leads to the final diagnosis. An accurate search for melanocytic tumors outside the central nervous system as a primary source is mandatory. PLMC has a better prognosis than PLM. There is no consensus regarding the adjuvant therapy, but patients with PLM should be given radiotherapy, chemotherapy, and immunotherapeutic approaches as immune checkpoint blockade after surgery. Communicating hydrocephalus is highly associated with PLM, but may occur in PLMC as well.
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Persistent primitive trigeminal artery (PPTA) is a rare abnormal carotid-basilar anastomosis. In rare circumstances, PPTA can be associated with some cerebrovascular anomalies, including arteriovenous malformation, intracranial aneurysm, carotid cavernous fistula, and moyamoya disease (MMD). ⋯ The pathogenesis of the coexistence of PPTA and MMD, PPTA and intracranial aneurysm, and MMD and intracranial aneurysm is still unknown. According to the literature, congenital factor and hemodynamic stress may play an important role in the formation of these vascular anomalies. In case of hypoplasia of the vertebrobasilar system, PPTA could also be used as a route for endovascular coiling for some specific aneurysms in the posterior circulation. To our knowledge, this is the first report of such a case.