Radical Surgery in a Neonate with Craniopharyngioma

Müller-Scholden, J.; Lehrnbecher, Thomas; Müller, Hermann L.; Bensch, Jürgen; Hengen, R; Sörensen, N.; Stockhausen, H. B. von

doi:10.1159/000055967

Cited by 66 publications

(30 citation statements)

References 16 publications

(22 reference statements)

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“…Craniopharyngioma represent 1.2–4% of all childhood intracranial tumors. In childhood and adolescence, its histological type is usually adamantinous, with cyst formation [3]; it also arises in adulthood, with peak incidence between the ages of 50 and 75, most often with a papillary histological type. Cases of craniopharyngioma are systematically documented by the German Paediatric Cancer Registry (Deutsches Kinderkrebsregister) in accordance with international guidelines [4].…”

Section: Epidemiology and Pathologymentioning

confidence: 99%

Childhood Craniopharyngioma

Müller

2008

Horm Res Paediatr

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108

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Background: Craniopharyngioma are embryogenic malformations of the sellar area. With an overall incidence of 0.5–2 new cases per million population per year, 30–50% of all cases occur in childhood. Overall survival rates are high. However, quality of life (QoL) is substantially reduced in many survivors due to sequelae such as extreme obesity caused by hypothalamic lesions. Methods: Based on retrospective analysis of 306 patients with childhood craniopharyngioma (HIT ENDO), we found that QoL was negatively related to hypothalamic involvement, tumor size and the number of neurosurgical interventions. Results: Irradiation had no significant impact on long-term QoL. The prospective surveillance of 98 patients in KRANIOPHARYNGEOM 2000 revealed frequent and early events in terms of tumor relapse after apparently complete resection (EFS: 0.60 ± 0.09 at 3 years) and tumor progression after incomplete resection (EFS: 0.22 ± 0.09). Conclusion: We conclude that radical surgery in patients with hypothalamic involvement has a major negative impact on long-term QoL. Innovative treatment strategies are warranted to improve QoL in these patients at risk. Accordingly, the appropriate time point of irradiation after incomplete resection will be analyzed in KRANIOPHARYNGEOM 2007.

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Section: Epidemiology and Pathologymentioning

confidence: 99%

Childhood Craniopharyngioma

Müller

2008

Horm Res Paediatr

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108

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“…Overall there are 0.5-2 new cases per million population occurring each year, 30-50% of which are children and adolescents [1]. The peak incidence is at age 5-10 years but they can occur at any age including infancy and pre and neonatal period [2]. Although the tumor itself is benign and the overall survival rate of patients is high [3], there is considerable morbidity even when the tumor can be completely resected [4][5][6][7].…”

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confidence: 99%

Melatonin Treatment in Obese Patients with Childhood Craniopharyngioma and Increased Daytime Sleepiness

Müller

Handwerker

Gebhardt

et al. 2006

Cancer Causes Control

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Craniopharyngioma is a rare dysontogenetic benign tumor. Patients frequently suffer from endocrine deficiencies, sleep disturbances and obesity due to pituitary and hypothalamic lesions. A self-assessment daytime sleepiness questionnaire (German version of the Epworth Sleepiness Scale [ESS]) was used to evaluate 79 patients with childhood craniopharyngioma. Because hypothalamic lesions may explain daytime sleepiness in craniopharyngioma patients, salivary melatonin and cortisol concentrations were examined in severely obese (BMI>or=4SD) and non severely obese (BMI<4SD) craniopharyngioma patients (n=79), patients with hypothalamic pilocytic astrocytoma (n=19), and control subjects (n=30). Using a general linear model procedure analyzing the influence of BMI and tumor diagnosis on diurnal salivary melatonin we found that morning salivary melatonin levels were related to BMI (F test: p-value=0.004) and tumor diagnosis (F-test: p-value=0.032). Also for nighttime salivary melatonin levels significant relations with BMI (p-value in F-test: <0.001) and tumor diagnosis (p-value in F-test: 0.025) were detectable. Melatonin concentrations in saliva of craniopharyngioma patients collected at nighttime or in the morning showed a negative correlation (Spearman's rho: -0.42; p=0.001; Spearman's rho: -0.31; p=0.020) with the patient's ESS score. Severely obese craniopharyngioma patients and severely obese hypothalamic tumor patients had similar patterns of melatonin secretion. Differences in terms of diurnal salivary cortisol concentrations were not detectable when patient groups and controls were compared. As decreased nocturnal melatonin levels were associated with increased daytime sleepiness, BMI and hypothalamic tumor diagnosis, we initiated an experimental melatonin substitution in 10 adult obese patients (5f/5m) with childhood craniopharyngioma. In all 10 patients with childhood craniopharyngioma the degree of daytime sleepiness significantly improved based on activity diaries, ESS, self assessment questionnaires and actimetry. We speculate that hypothalamic lesions might be responsible for both obesity and daytime sleepiness. As first experiences with experimental melatonin substitution were promising, further randomized double-blinded studies on the beneficial effects of melatonin substitution on daytime sleepiness and weight control in these patients are warranted.

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“…Kultursay et al [11] reported the surgical resection (done at 4 weeks of age) of a craniopharyngioma identified by fetal ultrasound and MRI. Muller-Scholden et al [13] reported the surgical management (done at 17 days of age) of a craniopharyngioma seen on fetal ultrasound and on CT done after delivery.…”

Section: Discussionmentioning

confidence: 99%

Congenital Craniopharyngioma: A Role for Imaging in the Prenatal Diagnosis and Treatment of an Uncommon Tumor

Kolen¹,

Horvai

Perry³

et al. 2003

Fetal Diagn Ther

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Ultrasonography and magnetic resonance imaging performed upon a male fetus at 32 and 36 weeks gestation, respectively, revealed a large suprasellar mass. A male newborn, delivered at 37 weeks, required ventilatory assistance at birth and subsequently developed myoclonic seizures, hypertension, and bradycardia. The intracranial mass was felt to be inoperable and the patient expired shortly after support was withdrawn. Autopsy results were consistent with a congenital craniopharyngioma. We discuss the differential diagnosis for this mass lesion based on prenatal imaging as well as distinguishing features on imaging studies that may aid in the prenatal diagnosis and treatment of this benign tumor.

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Radical Surgery in a Neonate with Craniopharyngioma

Cited by 66 publications

References 16 publications

Childhood Craniopharyngioma

Childhood Craniopharyngioma

Melatonin Treatment in Obese Patients with Childhood Craniopharyngioma and Increased Daytime Sleepiness

Congenital Craniopharyngioma: A Role for Imaging in the Prenatal Diagnosis and Treatment of an Uncommon Tumor

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