RESUMO
Itsenko-Cushing's disease is a rare, multisystem disease characterized by the presence of endogenous central hypercortisolism due to an ACTH-secreting brain tumor. The frequency of Itsenko-Cushing's disease in adulthood is 0.7-2.4 per 1 million population, and only 10% of all cases occur in childhood. The age of onset of the disease in children is on average 12.0-14.8 years. A typical manifestation of the disease in children, along with obesity and arterial hypertension, is a decrease in growth rates. The gold standard for diagnosing central hypercortisolism is MRI of the brain, however, the effectiveness of this method in children is only 50%. The main method of treatment is neurosurgical transnasal transsphenoidal removal of endosellar pituitary adenoma, which makes it possible to achieve remission in more than 65% of cases. This article describes a clinical case of Itsenko-Cushing's disease in a 6.5-year-old child with obesity, arterial hypertension, atypically «high¼ stature, average velocity and non-visualizable corticotropinoma. The article presents the stages of diagnostic search, the complexity of differential diagnosis and surgical treatment, the results of follow-up after the treatment and a brief review of the literature.
Assuntos
Adenoma , Síndrome de Cushing , Hipertensão , Hipersecreção Hipofisária de ACTH , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Obesidade , Hipersecreção Hipofisária de ACTH/complicações , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/cirurgiaRESUMO
Malignant pineal tumors (PT) tend to metastasize. In rare cases, this process is provoked by surgery. This paper describes four cases of iatrogenic metastasis of PT. Metastasis occurred: a) along the surgical approach path after tumor removal in 2 cases; b) along the biopsy cannula path; and c) along the ventriculoperitoneal catheter into the abdomen. The sources of metastases were pineoblastoma (n = 1), malignant teratoma (n = 1), germinoma (n = 1), and malignant germ-cell tumor of unknown genesis (n = 1). To prevent this complication due to high-grade PT, such as malignant germ-cell tumors and pineoblastomas, radiation of the whole brain, besides the sites of a tumor should be performed and, in some cases, in combination with chemotherapy.