A clinical Case and brief literature review of Icenko-Cushing’s Disease in a pediatric patient with atypical onset of the disease

• Published in: Problemy ėndokrinologii 2022-05, Vol.68 (4), p.92-101
• Main Authors: Tiulpakov, M. A., Bezlepkina, O. B., Nagaeva, E. V., Azizian, V. N., Lapshina, A. M.
• Format: Article
• Language: eng ; rus
• ISSN: 0375-9660; 2308-1430
• DOI: 10.14341/probl13102

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 «high» 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.