The diencephalon is a complex midline structure consisting of the hypothalamus, neurohypophysis, subthalamus, thalamus, epithalamus, and pineal body. Tumors arising from each of these diencephalic components differ significantly in terms of biology and prognosis. The aim of this comprehensive review is to describe the epidemiology, clinical symptoms, imaging, histology, and molecular markers in the context of the . We will also discuss the current management of each of these tumors.
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http://dx.doi.org/10.3389/fonc.2023.1180267 | DOI Listing |
Zh Vopr Neirokhir Im N N Burdenko
October 2024
Burdenko Neurosurgical Center, Moscow, Russia.
Pediatr Blood Cancer
December 2024
Department of Ophthalmology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Arch Argent Pediatr
October 2024
Clínica Universitaria Reina Fabiola, Córdoba, Argentina.
Russell's diencephalic syndrome is a set of signs and symptoms characterized by extreme weight loss, with no impairment of height or head circumference, without changes in intake or appetite. It is due to hypothalamic dysfunction associated with space-occupying lesions in this region. It is a rare cause of malnutrition in pediatrics, so its diagnosis is often delayed.
View Article and Find Full Text PDFEndocr Connect
October 2024
Department of Pediatric Neuro-oncology, Princess Máxima Center, Heidelberglaan, CS Utrecht, The Netherlands.
Objective: Children with a supratentorial midline low-grade glioma (LGG) may be at risk for impaired bone health due to hypothalamic-pituitary dysfunction, obesity, exposure to multiple treatment modalities, and/or decreased mobility. The presence of impaired bone health and/or its severity in this population has been understudied. We aimed to identify the prevalence and risk factors for bone problems in children with supratentorial midline LGG.
View Article and Find Full Text PDFMol Syndromol
August 2024
Division of Pediatric Neurology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Introduction: Diencephalic-mesencephalic junction dysplasia syndrome is a rare neurogenetic disorder reported to be caused by variants in several genes. Phenotypic presentation is characterized by clinical findings including developmental delay, hypotonia, spasticity, and dyskinetic movements in combination with distinctive imaging features on brain magnetic resonance imaging (MRI).
Methods: Whole exome sequencing was conducted to unveil the molecular etiology of patients presenting with neurological manifestations from two unrelated families.
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