Diagnostics, treatment, and follow-up in craniopharyngioma

Abstract

Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region, with up to half the 0.5-2.0 new cases per million population per year occur in children and adolescents. Diagnosis profile for pediatric and adult craniopharyngioma is characterized by a combination of headache, visual impairment, and polyuria/polydipsia, which can also include significant weight gain. In children, growth retardation, and/or premature puberty often occur later or postoperatively. Recommended therapy with favorable tumor localization is complete resection; with unfavorable tumor localization (optic nerve and/or hypothalamic involvement), consensus is still pending whether a limited resection followed by local irradiation is more prudent. Even though overall survival rates are high (92%), recurrences after complete resection and progressions after incomplete resection can be expected. Accordingly, a randomized multinational trial (KRANIOPHARYNGEOM 2007) has been established to identify optimal diagnosis, treatment (particularly the ideal time point of irradiation after incomplete resection), and quality of life strategies of this chronic disease - most notably the morbid hypothalamic obesity in ∼50% of long-term survivors. We report on craniopharyngioma origins, its pathological manifestations, and specific challenges these sequelae pose regarding diagnosis, treatment, and life-long multi-discipline quality of life management for both adult and childhood craniopharyngioma patients.

Keywords: brain tumors; craniopharyngioma; hypothalamus; irradiation; neurosurgery; obesity; pituitary; quality of life.

Figure 1

Manifestations before diagnosis of craniopharyngioma in children and adolescents. Frequency of occurrence of each manifestation before diagnosis (blue) and frequency of occurrence as the initial manifestation (red). The median time (month) from the appearance of each initial manifestation until diagnosis is indicated above each red column. In the overall group, the median time from the initial manifestation of disease until diagnosis was 12 months, with a range of 0.01–96 months. (Modified from Müller et al., , with the kind permission of Springer).

Figure 2

Degree of obesity in relation to the localization of childhood craniopharyngioma. In both patients craniopharyngioma (as indicated by arrow on MRI before surgery) could be completely resected. Both patients had complete hypopituitarism after surgery requiring endocrine substitution of all hypothalamic–pituitary axes. The patient depicted in (B) developed severe obesity due to hypothalamic lesions of suprasellar parts of craniopharyngioma (C). The patient depicted in (A) presented with a small tumor confined to the sellar region (D). After complete resection she kept normal weight without any eating disorders. (Modified from Müller et al., , with the kind permission of Springer).

Figure 3

Kaplan–Meier analyses of event-free survival rates (EFS) depending on the extent of resection among the 117 craniopharyngioma patients recruited in the trial KRANIOPHARYNGEOM 2000. (Modified from Müller et al., , with the kind permission of Karger).

Figure 4

Study design of KRANIOPHARYNGEOM 2007 (www.kraniopharyngeom.net; modified from Müller et al., , with the kind permission of Thieme).

Figure 5

Changes in body mass index (BMI SDS) during first 36 months after diagnosis of 117 childhood craniopharyngioma patients recruited in KRANIOPHARYNGEOM 2000 relative to the extent of surgical hypothalamic lesions (grade 0–2). The horizontal line in the middle of the box depicts the median. Edges of box mark the 25th and 75th percentile. Whiskers indicate the range of values that fall within 1.5 box-lengths. (Modified from Müller et al., , with the kind permission of BioScientifica).