Outcome of craniopharyngioma in children: long-term complications and quality of life

Abstract

Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic-pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow-up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health-related quality of life were assessed (medical follow-up, semi-structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late-treatment complications decreased quality of life for many long-time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health-related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents' ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours.

Hydrocephalus and hypothalamic involvement in pediatric patients with craniopharyngioma or cysts of Rathke's pouch: impact on long-term prognosis

“…Causes of late mortality include those directly related to the tumor or its treatment such as progressive disease with multiple recurrences, chronic hypothalamic insufficiency, hormonal deficiencies, cerebrovascular disease, and seizures (26,38,40,48). Other disease-related causes of mortality have been described, including decreased mineral bone density and non-alcoholic steatohepatitis, leading to liver cirrhosis in some cases (38,40). A recent review has reported substantial long-term morbidity with hypopituitarism, increased cardiovascular risk, hypothalamic damage, visual and neurological deficits, reduced bone health, and reduction in QoL and cognitive function (49).…”

Autonomic nervous system balance in children and adolescents with craniopharyngioma and hypothalamic obesity

“…One such cause is tumors and their treatment effects, including craniopharyngioma (3). Craniopharyngioma tumor histology is benign and long-term survival rates are high (4,5). Complications are also common, and both the tumor and the treatment can lead to significant long-term endocrinological and neurological morbidity.…”

Markedly Improved Glycemic Control and Enhanced Insulin Sensitivity in a Patient with Type 2 Diabetes Complicated by a Suprasellar Tumor Treated with Pioglitazone and Metformin

“…Similarly, patients with hypothalamic damage due to structural lesions (e.g., craniopharyngioma) or genetic lesions (e.g., Prader-Willi syndrome) may develop central obesity associated with ravenous appetite and insulin resistance (5)(6)(7)(8)(9)(10). It is proposed that the damaged or dysfunctional hypothalamus in these patients is insensitive to the effect of leptin (11).…”