RESUMO
Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal-radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.
Assuntos
Neoplasias Cerebelares/complicações , Hemorragia Cerebral/etiologia , Hemangioblastoma/complicações , Adulto , Fatores Etários , Idade de Início , Idoso , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/epidemiologia , Neoplasias Cerebelares/patologia , Angiografia Cerebral , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/epidemiologia , Hemorragia Cerebral/patologia , Ventrículos Cerebrais/patologia , Progressão da Doença , Embolização Terapêutica/efeitos adversos , Feminino , Hemangioblastoma/diagnóstico , Hemangioblastoma/epidemiologia , Hemangioblastoma/patologia , Humanos , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/epidemiologia , Hemorragia Subaracnóidea/epidemiologia , Hemorragia Subaracnóidea/etiologia , Tomografia Computadorizada por Raios XRESUMO
The authors report the case of a 23-year-old woman with café-au-lait spots and axillary and inguinal freckling who presented with a diploic chronic spontaneous hematoma of the left parietal bone. To the authors' knowledge, this case represents the first description of a diploic hematoma in a patient with stigmata of neurofibromatosis Type 1 unrelated to head trauma. Plain skull radiography showed an osteolytic lesion with well-circumscribed margins, corresponding to the hematoma, together with exuberant perilesional vascular markings. Angiography demonstrated an incidental aneurysm of the left supraclinoidal internal carotid artery and an unusual cortical venous drainage toward the diploic vessels. The blood flow of these vessels on the right hemicranium was sluggish and exhibited enlarged diploic venous lacunas. The authors hypothesize that the hematoma was formed by both an abnormal venous drainage toward the diploic vascular net, together with a vasculopathy that caused stenosis and obstruction of the normal drainage pathways from these vessels.