Fetal Craniopharyngioma: A Rare Prenatal Diagnosis

Adamantinomatous craniopharyngioma (ACP) is a benign epithelial tumor classically diagnosed in childhood and only exceptionally during fetal life. We report a case of prenatally diagnosed ACP at 30 weeks of gestation (WG). Routine third-trimester ultrasound (US) revealed a midline suprasellar brain mass with mixed, solid, and cystic components with calcifications. Fetal MRI confirmed the initial findings, suggesting the differential diagnosis of either teratoma or craniopharyngioma. The parents opted for a medical termination of pregnancy, performed under the current French ethics law at 31WG. Fetopathological examination demonstrated an isolated suprasellar tumor. Histopathology was consistent with the diagnosis of adamantinomatous craniopharyngioma. Including the present contribution, fewer than 20 prenatal diagnoses of craniopharyngioma have been reported so far. Notably, all fetal cases correspond to the adamantinomatous subtype and share similar radiological and histological features. This observation enhances the concept that the full morphological phenotype of ACP is already established during fetal life. Despite its benign nature, ACP may lead to severe long-term visual, endocrine, and neurodevelopmental morbidity. This case contributes to the better understanding of fetal ACP and highlights the importance of accurate prenatal diagnosis and fetopathological examination for establishing its diagnosis.