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

Abstract

Objective: Pediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke's pouch (CRP) frequently suffer disease-and treatment-related sequelae. We analyzed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP. Subjects and methods: Using retrospective analysis of patient records, presence of initial HY or HI was assessed in 177 pediatric patients (163 CP and 14 CRP). Twenty-year overall survival (OS) and progression-free survival (PFS), FC, and BMI were analyzed with regard to initial HY, degree of resection, or HI. Results: Of the 177 patients, 105 patients (103/163 CP and 2/14 CRP) presented with initial HY and 96 presented with HI. HY at diagnosis was associated (PZ0.000) with papilledema, neurological deficits, and higher BMI at diagnosis and during followup. OS, PFS, and FC were not affected by HY at initial diagnosis. HI at diagnosis (96/177) had major negative impact on longterm prognosis. Sellar masses with HI were associated with lower OS (0.84G0.04; PZ0.021), lower FC (PZ0.003), and higher BMI at diagnosis and last follow-up (PZ0.000) when compared with sellar masses without HI (OS: 0.94G0.05). PFS was not affected by HI or degree of resection. Conclusions: Initial HY has no impact on outcome in patients with sellar masses. OS and FC are impaired in survivors presenting with initial HI. PFS is not affected by HY, HI, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial HI to prevent further hypothalamic damage.

History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis

“…HI of CP has been reported as a major risk factor to impairment of long-term survival (7,8,14) and quality of life (8,21,22,23,24,25,26,27,28,29,30) in CP patients. The question of whether DOH before diagnosisspecifically whether prolonged interval between the first appearance of symptoms and the time of diagnosiscontributes to adverse sequelae and impaired survival had not been answered before this study.…”

“…Hydrocephalus at initial CP diagnosis has been recently reported as a clinical manifestation not associated with impaired outcome in terms of survival and FC (8). However, hydrocephalus did influence Table 3 Patients characteristics and outcome in terms of 10-year overall survival, 10-year progression-free survival, degree of obesity (BMI SDS (17) 9.8 (1.1-128.7) 0.02 y/n, yes/no; bold values and ranges indicate significant differences.…”

“…They show low-grade histological malignancy (WHO 8I) and frequently affect hypothalamic and pituitary regions due to their location. Hypothalamic involvement (HI) of CP, resulting in pathological patterns of eating behavior and obesity, is the most serious risk factor -its aforementioned manifestations impairing prognosis and quality of life in surviving patients (1,3,4,5,6,7,8,9,10,11,12,13,14,15). suspicion of childhood CP in the differential diagnosis process (5).…”

MANAGEMENT OF ENDOCRINE DISEASE: Childhood-onset craniopharyngioma: state of the art of care in 2018

“…The prognostic impact of an initial hydrocephalus is also still a matter of debate. Increased mortality due to primary hydrocephalus has been reported as well as a lack of association between hydrocephalus and mortality (28,38).…”

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

“…Follow-up studies of QoL in children after complete resection of CP revealed that QoL depends on the experience of the operating neurosurgeon [44,45,46]. Daubenbüchel et al [47] recently published that 20-year overall survival rates were significantly lower in CP patients with initial involvement of hypothalamic structures when compared with CP without hypothalamic involvement (Figure 3A,B).…”