Papillary Craniopharyngioma in an Adolescent Presenting with Bilateral Optic Atrophy and Endocrine Dysfunction

Dear Editor, Papillary craniopharyngioma is a rare benign intracranial tumor that predominantly affects adults and is exceedingly uncommon in adolescents. Craniopharyngiomas account for approximately 2%–5% of intracranial tumors and are classified as World Health Organization Grade I lesions arising from epithelial remnants of the craniopharyngeal duct.[1] We report a rare adolescent case to emphasize the clinical consequences of delayed recognition of visual and endocrine symptoms. An 18-year-old female presented with progressive bilateral visual impairment, chronic headache, and primary amenorrhea since the age of 13. Visual symptoms began insidiously five years prior, initially affecting the left eye and gradually progressing to bilateral temporal field loss. Headache increased in frequency and severity over time and was exacerbated by Valsalva maneuvers. The patient had never menstruated and exhibited no secondary sexual characteristics. On examination, visual acuity was 3/60 in the right eye and 1/300 in the left eye, with bitemporal hemianopia and bilateral optic disc atrophy. General examination revealed underdeveloped breasts and the absence of axillary and pubic hair. Brain magnetic resonance imaging demonstrated a heterogeneous solid–cystic intrasellar–suprasellar mass with peripheral calcification and fluid–fluid levels suggestive of intratumoral hemorrhage, causing compression of the optic chiasm and pituitary structures, consistent with craniopharyngioma [Figure 1]. Serum prolactin was markedly elevated (2074 ng/mL), consistent with pituitary stalk compression.Figure 1: Contrast-enhanced magnetic resonance imaging of the brain demonstrating an intrasellar–suprasellar lesion. The mass is isointense on T1-, T2-, and fluid-attenuated inversion recovery-weighted sequences, with mild post-contrast enhancement in the solid component and along the cystic margin. The lesion measures 2.0 cm × 3.0 cm × 2.4 cm (solid) and 2.3 cm × 2.8 cm × 3.4 cm (cystic). Axial images show (A) T1-weighted, (B) contrast-enhanced T1, and (C) T2-weighted sequences. The adenohypophysis and infundibulum are obliterated.Figure 2: Histopathological examination of the tumor demonstrating cells with round nuclei, moderate to marked pleomorphism, vesicular chromatin with coarse areas, and occasional hyperchromatic nuclei. The cytoplasm is chromophobic and granular, with some cells showing eosinophilic features. Mitoses are observed, and focal calcifications are present. Immunohistochemistry shows (A) AE1/3 positivity and (B) synaptophysin negativity.The patient underwent extended endonasal transsphenoidal subtotal tumor resection. Histopathological examination confirmed papillary craniopharyngioma [Figure 2]. Postoperatively, headache improved; however, visual deficits persisted due to established bilateral optic atrophy, highlighting irreversible optic nerve damage from prolonged compression. Adjuvant radiotherapy was planned, and dopamine agonist therapy was initiated. Papillary craniopharyngioma is strongly associated with the B-Raf proto-oncogene, serine/threonine kinase V600E mutation and typically occurs in adults, with adolescent cases reported only sporadically.[2,3] Clinical manifestations depend largely on tumor location and duration of compression. In this case, delayed diagnosis resulted in permanent visual sequelae despite appropriate surgical management. Hyperprolactinemia-related hypogonadism further contributed to delayed pubertal development through suppression of the hypothalamic–pituitary–gonadal axis signaling.[2] This case underscores the importance of early neuroimaging in adolescents presenting with progressive visual loss and endocrine abnormalities. Prompt multidisciplinary evaluation is crucial to prevent irreversible neurological and endocrine complications, even in rare tumor subtypes traditionally associated with adult populations. Ethics approval and consent to participate A letter to the editor does not require ethical committee approval and registration number. This report was purely an observation, and there was no harm to the patient. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.