MON-091 The Clinical and Endocrinologic Manifestations of Craniopharyngioma in Taiwanese Pediatric Population: One Medical Center Experience

Introduction: Craniopharyngioma is a rare tumor located in the suprasellar region. Due to the unique tumor location, hypothalamic-pituitary axis dysfunction is usually presented in these patients. The major symptoms are headache, visual impairment, increased intracranial pressure, and endocrinologic abnormalities. However, diagnosis is often delayed because of slow growth of the tumor and subtle symptoms. Our aims are to describe the clinical and endocrinologic manifestations before the surgical resection of craniopharyngioma in Taiwanese pediatric population. Method: We retrospectively reviewed 42 pediatric patients of craniopharyngioma in a tertiary medical center between 1996 to 2019. The clinical parameter including the initial presentation, age at diagnosis, height, weight, pituitary gland function, tumor location, time to diagnosis, and pathology reports are collected and analyzed. Results: A total of 42 patients (24 boys and 18 girls) were included in the study. The mean age at diagnosis was 8.6 years, ranging from 1 to 16-year-old. The average time to diagnosis was 7.7 months and 15.9 months for the patients with and without neurological signs, respectively. The most common initial presentation is headache (25/42, 60%), followed by visual impairment (20/42, 48%) and short stature (13/38, 34%). If short stature was the only initial presentation, 4 (4/7, 57%) of them were diagnosed of craniopharyngioma because they developed subsequent neurological deficits during clinic follow-up. Pituitary function tests showed that10 patients (10/25, 40%) had TSH deficiency and 8 patients (8/16, 50%) had central adrenal insufficiency. Image studies reported 26 patients with hypothalamic involvement and 4 patients without. All of 8 patients (8/37, 22%) with obesity had a tumor involving the hypothalamus. Pathology reports revealed 31 adamantinomatous type and only 1 papillary type. Conclusion: The study showed most (71 percent) of Taiwanese pediatric patients with craniopharyngioma presented with at least one endocrine dysfunction. Therefore, comprehensive pituitary evaluation for all patients with craniopharyngioma should be warranted. Furthermore, neurological signs and pituitary functions should both be monitored during the clinic follow-up for children with short stature or obesity in order to early diagnosis of craniopharyngioma.