Craniopharyngioma in pediatrics: diagnosis and treatment
Craneofaringioma en pediatría : diagnóstico y tratamiento.
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Craniopharyngiomas (CP) are rare tumors of low histological malignancy that arise along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary (PCP), differ in origin and distribution by age. ACPs are diagnosed with a bimodal incidence peak (5–15 years and 45–60 years), while PCPs occur exclusively in adults, mainly in the fifth and sixth decades of life. ACPs are generated by somatic mutations in CTNNB1, encoder of β-catenin and are predominantly cystic in appearance. PCPs have somatic mutations in BRAF V600E and are typically solid tumors. The clinical manifestations of endocranial hypertension, visual compromise or endocrine deficiencies should alert us to request images. The treatment generally includes neurosurgery and radiotherapy (RT); Intracystic chemotherapy is used in monocystic ACP. Although long-term survival is high, quality of life and neuropsychological function are frequently affected, due to the anatomical proximity to the optic chiasma, the hypothalamus and the pituitary gland. Hypothalamic involvement by the tumor and injuries secondary to treatment often result in hypothalamic obesity and neuropsicosocial deficits.
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