Adamantinomatous craniopharyngioma of cerebellopontine angle in association with Gardner’s syndrome

Abstract

Background. Craniopharyngiomas are benign epithelial tumors. According to embryogenetic theory, these neoplasms develop from the remains of the cells of Rathke’s pouch, which connects the cavities of the primary oral tube with the pituitary gland in the embryonic period. The metaplastic theory assumes the occurrence of craniopharyngiomas as a result of metaplasia of epithelial cells in the chiasmal-sellar region.Objective. To present a clinical case of ectopic adamantium-like craniopharyngioma of the right cerebellopontine angle associated with Gardner’s syndrome.Design and methods. The analysis of SCT, MRI, histological, histochemical and other special studies, as well as data from four other similar observations published in the literature, was carried out.Results. We describe a case of a rare case of ectopic adamantium-like craniopharyngioma of the right cerebellopontine angle associated with Gardner’s syndrome (familial adenomatous polyposis, multiple skull osteomas and soft tissue tumors) diagnosed in a 27-year-old patient. It is noted, while inexplicable, the correlation of craniopharyngiomas in patients with Gardner’s syndrome in the region of the cerebellopontine angle.Conclusions. In the case of patients with adamantine-like craniopharyngiomas, the including of collection of a family history and special studies to exclude adenomatous intestinal polyposis and Gardner syndrome in the complex of treatment and diagnostics id highly recommended.