RESUMO
Craniopharyngiomas are rare hypothalamic-pituitary tumors found in young children, adolescents and adults, and their multidisciplinary management required, calls for consistent practices for practicioners, patients and families. The French Endocrine Society and French Society for Pediatric Endocrinology & Diabetes enlisted and coordinated adult and paediatric endocrinologists, neurosurgeons, pathologists, radiotherapists as well as psychologists, dieticians and a patient association, to draft a reference document on this severe disease. The management of craniopharyngiomas remains complex due to their aggressive nature, invasive behavior, and propensity for recurrence, requiring a sequential and measured therapeutic approach and follow-up in expert centers. Although patient survival rates are high, the consequences of both the tumor and its treatment can lead to serious comorbidities and impaired quality of life, particularly in those patients with lesional hypothalamic syndrome. Recent advances have allowed the two described tumor types - papillary and adamantinomatous - to be associated with distinct molecular signatures, specific pathophysiological mechanisms and ipso facto, distinct therapeutic approaches, including innovative medications for hyperphagia, that will continue to evolve. This consensus statement covers all stages in the management of patients with craniopharyngioma, from diagnosis to therapeutic strategies including the long-term follow-up.
RESUMO
The spontaneous occlusion of brain arteriovenous malformations (bAVMs) is a rare event, particularly for unruptured ones. Associated factors include single-venous drainage and small nidus size. Most of the previously reported cases were ruptured bAVMs. We report the case of a middle-aged male patient with an unruptured, rolandic, left-sided bAVM associated with a 30-year history of refractory epilepsy. We documented the spontaneous thrombosis of the venous drainage of the AVM without any sign of bleeding. Finally, we underline the difference between ruptured-induced occlusion and truly spontaneous thrombosis of the bAVMs.