Anika Hoffmann1, Svenja Boekhoff1, Ursel Gebhardt1, Anthe S Sterkenburg2, Anna M M Daubenbüchel2, Maria Eveslage1, Hermann L Müller3. 1. Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsInstitute of Biostatistics and Clinical ResearchUniversity of Münster, Münster, Germany. 2. Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsInstitute of Biostatistics and Clinical ResearchUniversity of Münster, Münster, Germany Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsInstitute of Biostatistics and Clinical ResearchUniversity of Münster, Münster, Germany. 3. Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsInstitute of Biostatistics and Clinical ResearchUniversity of Münster, Münster, Germany mueller.hermann@klinikum-oldenburg.de.
Abstract
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.
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.
Authors: Sergey Gorelyshev; Alexander N Savateev; Nadezhda Mazerkina; Olga Medvedeva; Alexander N Konovalov Journal: Adv Tech Stand Neurosurg Date: 2022
Authors: Marco Losa; Pietro Mortini; Alberto Luigi Gallotti; Lina Raffaella Barzaghi; Luigi Albano; Marzia Medone; Filippo Gagliardi Journal: Pituitary Date: 2021-07-08 Impact factor: 4.107