Craniopharyngioma in children: Report of three cases and review of the literature

Abstract

Craniopharyngioma is a rare benign epithelial tumor, originating in the pituitary stem or pituitary gland and developing in the sellar and / or suprasellar region. Of embryonic origin, craniopharyngiomas are considered to develop from epithelial remains of Rathke's pouch. It affects children as well as adults. All ages combined, craniopharyngiomas represent between 3 to 4% of intracranial tumors worldwide, or 0.5 to 2 new cases per year and per million inhabitants. In children, they represent 10% of all intracranial tumors with a peak frequency between 7 and 13 years and a predominance of men. Although benign, this tumor remains a serious pathology because of the frequent visual, endocrine, neuro-intellectual sequelae and the risk of recurrence that it entails, involving the visual and vital functional.The management of this pathology is multidisciplinary and involves several modalities such as surgery, radiotherapy and medical treatment of hormonal deficits very often associated. We report in this article three cases of boys aged 9, 10 and 12; treated for symptomatic craniopharyngiomas with a favorable clinical and radiological outcome after partial surgical excision and adjuvant external radiotherapy. The aim of this article is to review the epidemiological, diagnostic, therapeutic and evolutionary aspects of these benign tumors of the sellar and / or suprasellar region, with an emphasis on the interest of radiotherapeutic treatment.