Craniopharyngioma in adults and children: a study of 122 surgical cases

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.

Section: Discussionsupporting

confidence: 81%

Section: European Journal Of Endocrinologymentioning

confidence: 99%

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

Daubenbüchel

Hoffmann

Gebhardt

et al. 2015

“…The clinical combination of headache, visual impairment, decreased growth rate, and polydipsia/polyuria should arouse suspicion of childhood CP in the differential diagnosis (1,5,14,16,29,42,44,45,46,47,48,49,50,51,52). In our study, the combination of headache and growth failure (18%) was most frequent.…”

Section: Clinical Studymentioning

confidence: 82%

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

Hoffmann

Boekhoff

Gebhardt

et al. 2015

Objective: Childhood craniopharyngiomas (CP) are often diagnosed after a long duration of history (DOH). Tumor size, hypothalamic involvement (HI), and obesity are associated with reduced overall survival (OS) and functional capacity (FC). The effect of DOH and specific symptoms in history on presentation at initial diagnosis and long-term prognosis are unknown. Design: Retrospective analysis of patients' records and prospective longitudinal follow-up. Methods: Histories of 411 CP patients recruited in HIT Endo, KRANIOPHARYNGEOM 2000 were retrospectively evaluated for DOH, symptoms, and characteristics. The effect of specific manifestations and DOH on clinical presentation and tumor characteristics at time of initial CP diagnosis and long-term outcome were analyzed. Main outcome measures were 10-year OS and progression-free survival (PFS), FC, and BMI during longitudinal follow-up. Results: Median DOH was 6 months (range: 0.1-108 months) and correlated with age at diagnosis. Tumor size, HI, degree of resection, and BMI at diagnosis were not related to DOH. In multivariate analysis adjusted for age at diagnosis, only hydrocephalus was found to have a relevant influence on DOH. Visual and neurological deficits were associated with larger initial tumor size and impaired 10-year OS. Weight gain and growth failure were observed with longest DOH. PFS and FC were not related to any specific symptom. Endocrine deficits at diagnosis were associated with long DOH. Conclusions: CP is frequently diagnosed after long DOH, especially in older children. However, DOH was not associated with tumor size, HI, survival, or FC. Visual and neurological deficits necessitate rapid diagnostic workup.

“…Nearly half of craniopharyngiomas are greater than 3 cm in diameter at diagnosis (12,14). In patients with larger tumours, who have hydrocephalus or hypothalamic involvement and undergo radical resection, there is a high likelihood of hypothalamic morbidity (O50%), operative mortality (2.5-10%) and late mortality (11-28%) (15,16). Of relevance to the subject of this case presentation, hypothalamic obesity is associated with the extent of the neurosurgical procedure.…”

Section: Discussionmentioning

confidence: 99%

‘Do no harm’: management of craniopharyngioma

Gleeson¹,

Amin²,

Maghnie

2008

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