Long term sequelae of pediatric craniopharyngioma - literature review and 20 years of experience

Abstract

Craniopharyngioma are rare histologically benign brain tumors that develop in the pituitary-hypothalamic area. They may invade nearby anatomical structures causing significant rates of neurological, neurocognitive, and endocrinological complications including remarkable hypothalamic damage. Information regarding long term implications of the tumors and treatment in the pediatric population is accumulating, and treatment goals appear to be changing accordingly. In this review we aim to present data regarding long term complications of craniopharyngioma in children and adolescents and our experience from a large tertiary center. Hypothalamic dysfunction was noted to be the most significant complication, adversely affecting quality of life in survivors. Obesity, fatigue, and sleep disorders are the most notable manifestations of this dysfunction, and treatment is extremely difficult. Changes in management in recent years show a potential for improved long term outcomes; we found a trend toward less aggressive surgical management and increasing use of adjuvant treatment, accompanied by a decrease in complication rates.

Keywords: child; complications; craniopharyngioma; hypothalamic obesity; neurocognitive; pituitary; recurrence; sleep.

Figure 1

Trends in the surgical approach to craniopharyngioma 1975–2011. Prevalence of gross total resection (GTR – blue), subtotal resection (STR – red), and cyst decompression (Decompression – green) surgery as the primary treatment for pediatric craniopharyngioma in our institution over more than three decades; 1975–1989 (Hoffman et al., 1992), 1990–2001 (Ahmet et al., 2006), 2001–2011.

Figure 2

Prevalence of complications following Cp treatment in the past two decades. Prevalence of hypopituitarism (Hypopit), diabetes insipidus (DI), and obesity between the years 1990–2001 (Ahmet et al., ; blue) and 2001–2011 (red). Obesity (Ob) defined as BMI > 95th percentile for age and gender. Severe obesity defined as BMI > 99th percentile for age and gender or BMI > 40 kg/m² if more than 18 years old); Severe Ob/Ob – the percent of obese patients with severe obesity.

Figure 3

Features of the metabolic syndrome in patients with craniopharyngioma and control subjects.