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Craniopharyngioma: presentation and endocrine sequelae in 36 children
L de Vries1, L Lazar, M Phillip
1Institute for Endocrinology and Diabetes, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel. liatd@clalit.org.il
Insights
Craniopharyngioma often presents with non-endocrine symptoms in children, delaying diagnosis. Endocrinopathies like diabetes insipidus are common, especially delayed puberty in adolescents, impacting growth outcomes.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Neuro-oncology
Background:
- Craniopharyngioma is a rare brain tumor affecting the pituitary gland and surrounding structures.
- Endocrine dysfunction is a known complication, but its presentation and impact on growth are not fully characterized by age.
Purpose of the Study:
- To analyze the clinical presentation of craniopharyngioma in children based on age.
- To evaluate the impact of craniopharyngioma on height and body mass index (BMI) outcomes.
- To determine the prevalence of specific endocrinopathies, including diabetes insipidus and pubertal disturbances.
Main Methods:
- Retrospective study of 36 children diagnosed with craniopharyngioma.
- Analysis of presenting symptoms, focusing on age-related differences.
- Assessment of growth parameters (height standard deviation score - SDS) and BMI SDS at diagnosis and follow-up.
Main Results:
- Common presenting symptoms include headache (51.4%), vomiting (31%), and visual disturbances (22.9%).
- Endocrinopathies such as diabetes insipidus (actual rate 52%) and growth deceleration were frequently overlooked.
- Delayed puberty was observed in all age-appropriate adolescents, and final height SDS was comparable to target height SDS, with BMI SDS influenced by parental BMI SDS.
Conclusions:
- Endocrinopathies are prevalent in pediatric craniopharyngioma but rarely the primary reason for medical referral.
- Prepubertal children often present with non-endocrine symptoms, while adolescents are frequently referred due to delayed puberty.
- Diabetes insipidus may be more common than previously reported, and BMI is influenced by parental factors when hypothalamic obesity is excluded.
Abstract:
We studied the clinical presentation by age of 36 children with craniopharyngioma, and outcome by height and body mass index (BMI). Presenting symptoms included headache (51.4%), vomiting (31%), visual disturbances (22.9%), polyuria and/or polydipsia (17.1%), delayed puberty (19.4%), short stature (13.8%), and precocious puberty (2.7%). Growth deceleration was overlooked, as was diabetes insipidus (actual rate, 52% for both). Delayed puberty was observed in all patients of appropriate age. Mean height standard deviation score (SDS) at admission was significantly lower than mean target height SDS (p = 0.004), while mean final height SDS was similar (p = 0.14). BMI SDS at last follow-up was similar to mean parental BMI SDS. We conclude that although endocrinopathies are present in most patients with craniopharyngioma, they are rarely the reason for referral. While affected prepubertal children have non-endocrine complaints, most adolescents are referred because of delayed puberty. Diabetes insipidus may be more prevalent in craniopharyngioma than previously reported. When patients with hypothalamic obesity are excluded, mean BMI SDS remains within normal range and is influenced mostly by parental BMI SDS.