Primary Amenorrhea Revealing Adamantinomatous Craniopharyngioma Associated With Central Hypogonadotropic Hypogonadism in an Adolescent Girl: A Case Report.
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Adamantinomatous craniopharyngioma is an uncommon benign epithelial tumor arising from remnants of Rathke's pouch within the sellar-suprasellar region. Although patients typically present with headache, visual impairment, growth retardation, or multiple endocrine abnormalities, primary amenorrhea as the initial clinical presentation is unusual, particularly in the absence of neurological symptoms. We report the case of a 15-year-old adolescent girl who presented with primary amenorrhea and delayed pubertal development without headache, visual complaints, or other focal neurological deficits. Endocrine evaluation revealed central hypogonadotropic hypogonadism, raising suspicion of hypothalamic-pituitary axis dysfunction. Magnetic resonance imaging of the brain demonstrated a well-defined multilobulated, predominantly cystic sellar-suprasellar lesion measuring approximately 2.1 cm × 2.2 cm × 2.3 cm, containing a small enhancing solid component and imaging features suggestive of calcification. The lesion extended superiorly to the floor of the third ventricle and compressed the optic chiasm, with imaging findings highly suggestive of adamantinomatous craniopharyngioma. The patient was initially evaluated in the gynecology department and was subsequently referred to pediatric endocrinology. Following endocrine and radiological evaluation, multidisciplinary management involving pediatric endocrinology and neurosurgery was undertaken. This case highlights the importance of considering hypothalamic-pituitary lesions in adolescents presenting with primary amenorrhea and central hypogonadotropic hypogonadism, even in the absence of neurological manifestations. Early endocrine evaluation followed by timely neuroimaging is crucial for establishing the diagnosis, preventing irreversible visual and hypothalamic complications, and facilitating appropriate multidisciplinary management. This report emphasizes that primary amenorrhea may represent the earliest manifestation of adamantinomatous craniopharyngioma and underscores the importance of maintaining a high index of suspicion in adolescents with unexplained delayed puberty.