Craniopharyngioma and hypothalamic injury

Abstract

Purpose of reviewHypothalamic alterations, pathological or treatment induced, have major impact on prognosis in craniopharyngioma patients mainly because of consequent hypothalamic obesity. Recent insight in molecular genetics, treatment strategies, risk factors and outcomes associated with hypothalamic obesity provide novel therapeutic perspectives. This review includes relevant publications since 2013.Recent findingsRecent findings confirm that alterations in posterior hypothalamic areas because of tumour location and/or treatment-related injuries are associated with severe hypothalamic obesity, reduced overall survival and impaired quality of life in long-term survivors of childhood-onset craniopharyngioma. However, eating disorders are observed because of hypothalamic obesity without clear disease-specific patterns. Treatment options for hypothalamic obesity are very limited. Treatment with invasive, nonreversible bariatric methods such as Roux-en-Y gastric bypass is most efficient in weight reduction, but controversial in the paediatric population because of medical, ethical, and legal considerations. Accordingly, treatment in craniopharyngioma should focus on prevention of (further) hypothalamic injury. Presurgical imaging for grading of hypothalamic involvement should be the basis for hypothalamus-sparing strategies conducted by experienced multidisciplinary teams.SummaryUntil a nonsurgical therapeutic option for hypothalamic obesity for paediatric patients is found, prevention of hypothalamic injury should be the preferred treatment strategy, conducted exclusively by experienced multidisciplinary teams.

The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres

“…The rationale for RT in the primary treatment of craniopharyngioma is to stabilise residual disease after PR and avoid the unfavourable morbidity and mortality after CR (41). Our findings are in agreement with observations that the combination of conservative surgery and RT achieves tumour control that is similar to CR alone (29,31,32).…”

“…The significant increase in rates of hypothalamic morbidity from presentation to last follow-up in Group B but not in Group A might be explained by greater awareness and documentation of this complication in recent times. Müller and coworkers have highlighted the prevalence, prognostic indicators and sequelae of hypothalamic involvement from their landmark studies (31). This includes long-term results from the largest cohort of survivors from the multi-centre HIT-Endo registry (32).…”

Obesity and cahexia as the first manifestations of craniopharingioma

“…Оно выявляется в 30% случаев на момент диагностики КФ, но чаще развивается вследствие ее хирургического лечения, достигая в некоторых сериях 60-85% [10,11]. Риск развития ГО после операции по поводу КФ опреде-ляется, в первую очередь, степенью вовлечения гипота-ламуса в патологический процесс [12][13][14][15]. Результатом гипоталамического повреждения могут стать не только стремительное увеличение веса и развитие гиперфагии, но и вегетативная дисфункция, нарушение циркадных ритмов и терморегуляции, психические расстройства.…”

Zmiany torbielowate okolicy siodłowo-nadsiodłowej — diagnostyka i leczenie

“…Just as in Rathke's cleft cysts, vision impairment (62-84%), headache, and endocrine dysfunction (52-87%) occur and the symptom characteristics are not different from those observed in Rathke's cleft cyst. However, symptoms caused by compression of hypothalamic structures, including secondary amenorrhoea, obesity, circadian rhythm disturbances, and cardiovascular disorders, are much more frequent than in patients with Rathke's cleft cyst [29][30][31]. They are difficult to treat, and their incidence and severity may increase during therapy from approx.…”