Elsevier

Pediatric Neurology

Volume 50, Issue 1, January 2014, Pages 4-10
Pediatric Neurology

Topical Review
A Review of Childhood and Adolescent Craniopharyngiomas With Particular Attention to Hypothalamic Obesity

https://doi.org/10.1016/j.pediatrneurol.2013.09.003Get rights and content

Abstract

Background

Although craniopharyngiomas are considered “benign” neoplasms by the World Health Organization classification, these tumors may create significant morbidity and mortality in patients. Hypothalamic obesity is a frequent complication of craniopharyngiomas and is refractory to current management options.

Patients/Methods

We reviewed 24 cases of craniopharyngiomas treated from 1992 to 2010 in patients <18 years of age regarding clinical presentation, neuroimaging, recurrence, morbidity, and mortality, with particular attention to hypothalamic obesity.

Results

Our cohort conformed to published data in regard to neuroimaging characteristics, and clinical findings in the areas of endocrine, visual, neurological, neurobehavioral, and hypothalamic domains. At last follow-up, 53% of our patients were overweight (8%) or obese (46%). Only 25% of our patients had a healthy body mass index. Contrasting these data with body mass indices at diagnosis, where 21% of patients were overweight and 17% were obese, we found that there was a significant trend towards obesity over time. A significant portion of our mortality appears to be related to complications of obesity. The Native American population in Arizona appears to have a statistically greater incidence of obesity in childhood. Despite our small sample size, 75% of our Native Americans were obese at last follow-up and accounted for 50% of the mortality.

Conclusion

Hypothalamic obesity is a significant complication of craniopharyngiomas associated with increased mortality. The development of hypothalamic obesity is influenced by premorbid obesity, genetics, and therapy received, specifically radiation. Because of the intractability of hypothalamic obesity, improved understanding of neuroendocrine mechanisms, genomics, and newer antiobesity medications will be necessary to curb this significant complication.

Introduction

Craniopharyngiomas are benign cystic epithelial tumors of the sellar or suprasellar region and are the most common nonglial pediatric brain tumor.1 These tumors account for 6-15% of intracranial tumors in childhood and adolescence and account for 80% of neoplasms disrupting the hypothalamic-hypophyseal pathway.2, 3 Patients present with various symptoms and signs that are related to the size and location of the tumor. Because craniopharyngiomas originate adjacent to delicate structures, significant complications from the tumor or treatment may occur, including visual loss, neurological and behavioral deficits, endocrinopathies, cerebral vascular disease, hypothalamic dysfunction, or a syndrome of hypothalamic obesity. The Centers for Disease Control and Prevention defines obesity as having a body mass index (BMI) greater than the 95th percentile for age, overweight as being 85th-95th percentile for age, and underweight as <5th percentile for age. Hypothalamic obesity occurs in 60-80% of cases.4, 5, 6 The National Cancer Database showed survival rates for children <20 years of age were 86% at 2 years postdiagnosis and 80% at 5 years postdiagnosis.7 We reviewed 24 patients with craniopharyngiomas with regard to recurrence, morbidity, mortality, and the impact of obesity on outcome.

Section snippets

Patients and Methods

After obtaining institutional review board approval, we retrospectively reviewed the charts of 24 children diagnosed with craniopharyngiomas who were treated at our institution between 1992 and 2010. Their sex, ethnicity, age at diagnosis, weight and height, course of treatment, follow-up, and morbidity/mortality were recorded. BMI was calculated and percentiles were obtained using the Centers for Disease Control and Prevention sex-specific growth charts from May 2000. Magnetic resonance scans

Demographics

In our cohort, 12 of the 24 patients were male (50%). Our ethnic demographics were 13 white, 7 Latino, and 4 Native American. The mean age at diagnosis was 10.6 years (range 2-16 years). The mean follow up was 5.03 years (range 1 month to 14 years). Specific demographics for all 24 patients are shown in Table 1.

Neuroradiology

All 24 (100%) patients had a cystic component to their tumor. Seventeen (70%) of the patients had an associated solid component, and 4 (16%) had additional calcifications. Eleven

Discussion

Craniopharyngiomas are tumors arising from remnants of squamous epithelium of the craniopharyngial duct. The term craniopharyngioma was first introduced by Harvey Cushing in 1932.8 These tumors are considered benign neoplasms with biologic behavior consistent with Grade I tumors by World Health Organization classification. Although long-term survival for patients with pediatric craniopharyngiomas has been as high as 80-90%, tumor and treatment related morbidity is significant.9 Because of their

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