‘Do no harm’: management of craniopharyngioma

Abstract

Craniopharyngioma management is challenging. Although histology is benign, the tumour can be clinically aggressive with local invasion and frequent recurrences. Extensive morbidity may be present at diagnosis and furthermore, occurs as a consequence of neurosurgery and radiotherapy. Hypothalamic symptoms can have a devastating effect on quality of life and may reduce life expectancy. This case highlights both the challenge of managing hyperphagia and morbid obesity and the importance of initial treatment preserving existing hypothalamic function and the need to avoid tumour recurrence and further surgery.A 11-year old boy presented with hydrocephalus secondary to a craniopharyngioma (he had visual failure and hypopituitarism but few hypothalamic symptoms). He underwent radical resection followed by radiotherapy. Following this treatment, he developed psychological and behavioural problems and hyperphagia. Weight gain in the first year (an increase from C1.4 to C3.7 S.D.) resulted in poor mobility and a fall which caused a slipped femoral epiphysis. In the next year, there was a 6-month period of unexpected weight loss (C4.2 to C3.8 S.D.) that culminated in emergency treatment for diabetic ketoacidosis secondary to severe insulin resistance. He developed a left hemiplegia, and a subsequent cerebral angiogram identified multiple stenoses of the Circle of Willis with a Moyamoya appearance secondary to radiotherapy. Weight gain has continued (C3.8 to C5.5 S.D.) so that bariatric surgery is a management option.European Journal of Endocrinology 159 S95-S99

Increased Wingless ( Wnt ) signaling in pituitary progenitor/stem cells gives rise to pituitary tumors in mice and humans

“…Therefore, total resection of the tumor is not always possible without damage to vital surrounding structures such as the hypothalamus and optic chiasm. In these children, radical excision is associated with unacceptable morbidity and mortality, but subtotal resection (without further adjuvant radiotherapy) predisposes them to a high (>60%) 3-y recurrence risk and further hypothalamic and visual compromise (23,24). Although a conservative surgical approach leaving residual tumor with adjuvant radiotherapy has been adopted recently, tumor recurrence is still high.…”

“…Activating mutations in the gene encoding β-catenin catenin (cadherin-associated protein β1; CTNNB1) have been identified in the pediatric form of human craniopharyngioma (adamantinomatous craniopharyngioma, ACP) (20)(21)(22). These tumors are rare but are the most common nonneuroepithelial intracerebral tumors in children and are potentially devastating because of their highly infiltrative behavior and tendency to recur after surgical resection (23,24). Whether the identified mutations in CTNNB1 are causative of human ACP remains unknown.…”

Magnetic resonance imaging–graded hypothalamic compression in surgically treated adult craniopharyngiomas determining postoperative obesity

“…3,20,29,30 While it is becoming well accepted that there is an association with hypothalamic compression and perhaps sur- gical management, both may contribute to postoperative obesity in patients with tumors of this region, and this assumption cannot be directly drawn to include adults, 2 as growth and development are accompanied by significant changes in the hormone milieu in children, in contrast to adults. 9 Unfortunately, we have found in adult patients at risk for postoperative obesity that this perioperative morbidity is a real concern. Furthermore, as we become more aware of this complication, it becomes increasingly important that we focus some of our postoperative care on counseling our patients about its occurrence, detecting this complication, and preventing it.…”

Comparison of energy expenditure, body composition, metabolic disorders, and energy intake between obese children with a history of craniopharyngioma and children with multifactorial obesity

“…After surgery, obesity is present in up to 52% of patients, some of whom have severe difficulties to control their eating habits, and is refractory to treatment (6). Studies analyzing risk factors for the development of obesity in childhood craniopharyngioma patients show that an increase of body mass index (BMI) from the moment of diagnosis is predictive for the development of obesity after surgical removal of the tumor.…”