High prevalence of long‐term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma

Abstract

Craniopharyngioma is associated with excessive long-term multisystem morbidity and mortality, especially in female patients, despite a high cure rate. These observations indicate that dedicated long-term follow-up of these patients is required. The purpose of the follow-up should be: first, to look for recurrences and to ensure appropriate endocrine replacement, especially oestrogen replacement in premenopausal females; and second, to achieve intensive control of glucose, lipids, blood pressure and weight, as in any other patient with increased risk for cardiovascular disease.

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

“…The role of sex as a prognostic factor is not established; some authors report a higher mortality among females (28,29), but others have not found any sex differences (13,15,16). One of the two studies reporting higher mortality rates in females suggested a possible role of estrogen deficiency (29), but the other study did not consider that unsupplemented gonadal insufficiency had a significant impact on enhanced mortality (28).…”

“…The OS rates (which reflect effect of multiple treatments) described in an exclusively pediatric series ranged from 83 to 96% at 5 years (14, 15, 17, 30, 31, 32, 33) and 65 to 100% at 10 years (15, 26, 30, 31, 32, 34, 35, 36, 37, 38, 39, 40, 41), averaging 62% at 20 years (42). In adults or a broad age-range population (adults and children) series, the OS rates ranged from 54 to 96% at 5 years (13,16,25,29,32,43,44,45,46), 40 to 93% at 10 years (13,16,25,28,29,32,43,44,45,46), and 66 to 85% at 20 years (29,45,46). The lower limits of survival rates usually reflected data from earlier series that occurred before modern advances in microsurgery, neuroimaging, and radiotherapy.…”

“…CPs, although histologically benign, are associated with significant mortality, with reported overall mortality rates three to five times higher than those of the general population (28,29). The OS rates (which reflect effect of multiple treatments) described in an exclusively pediatric series ranged from 83 to 96% at 5 years (14, 15, 17, 30, 31, 32, 33) and 65 to 100% at 10 years (15, 26, 30, 31, 32, 34, 35, 36, 37, 38, 39, 40, 41), averaging 62% at 20 years (42).…”

Clinical characteristics and effects of GH replacement therapy in adults with childhood-onset craniopharyngioma compared with those in adults with other causes of childhood-onset hypothalamic–pituitary dysfunction

“…Due to the propensity for local recurrence of the tumour, more extensive and/or multiple surgeries and/or radiotherapy is frequently advocated, which often results in hypothalamic-pituitary dysfunction in these patients. Hypothalamic damage has been implicated as the main risk factor for obesity in patients with COCP (7), and the increased cardiovascular risk and poor QoL have been reported to be related to both obesity and GHD (3,8) in these patients. In addition, GHD due to COCP has been reported to increase the risk of metabolic syndrome (9).…”

“…Although histologically benign, most patients with craniopharyngiomas (85-90%) have significant pituitary hormone deficits (2, 3) with growth hormone (GH) deficiency (GHD) being the most common (54-100%) (4), and have decreased quality of life (QoL) with significant psychosocial and neurocognitive impairment (3). Previous studies have reported that patients with craniopharyngiomas not only have three-to fivefold higher morbidity and mortality rates compared with the general population (3,5), but also have higher morbidity and mortality rates compared with patients with other causes of hypopituitarism, namely those with non-functioning pituitary adenomas (6).…”