Craniopharyngioma – A childhood and adult disease with different characteristics

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Background

Craniopharyngiomas are benign, partly cystic embryogenic malformations of the sellar region, presumably derived from Rathke cleft epithelium. With an overall incidence of 0.5–2 new cases per million population per year, approximately 30-50% of all cases represent childhood craniopharyngioma. Typical manifestations at primary diagnosis are headache, visual impairment, polyuria/polydypsia, growth retardation, and weight gain.

One of the biggest challenges in treating craniopharyngioma is identifying the best candidates for the radical versus the conservative approach. It appears there is a trend towards radiotherapy in centers with past prevalent surgical approaches, and towards more radical surgical treatment strategies in centers historically conservative-oriented. There are current prospective studies underway on a national and multinational level to adopt strategies tailored to risk factors for morbidity and QOL. Therapy of choice in patients with favorable tumor localization is total resection with the intention to maintain optical nerve and hypothalamic-pituitary functions. In patients with unfavorable tumor localization (hypothalamic tumor involvement), a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%). Recurrences after complete resection and progressions of residual tumor after incomplete resection are anticipated subsequent events after primary surgery.

In clinical practice, the timing of postoperative residual tumor irradiation is both unclear and inconsistently regarded. Some favor immediate postoperative irradiation in the event of life-impairing clinical conditions, proactively preventing tumor progression. On the other hand, some favor a wait-and-see procedure, delaying irradiation in order to reduce both its necessity and the negative consequences associated with radiation therapy. Inarguably, immediate postoperative irradiation significantly delays tumor progression. However, progression-contingent irradiation has proved effective, as overall survival is statistically unaffected by this wait-and-see strategy. Accordingly, the appropriate time point of irradiation after incomplete resection is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007). Quality of life is substantially reduced in appr. 50% of long-term survivors due to sequelae, notably extreme obesity due to hypothalamic involvement. Due to the lack of satisfactory long-term treatment modalities for hypothalamic sequelae, further research on molecular characteristics of craniopharyngioma, pathophysiology of hypothalamic disorders, and pharmaceutical agents to treat hypothalamic obesity are warranted.

Risk-adapted surgical strategies at initial diagnosis should aim at a maximal degree of resection, respecting the integrity of optical and hypothalamic structures in order to prevent severe sequelae and therein minimizing consequences that could negative impact patient QOL. Because initial hypothalamic tumor involvement has an apriori effect on the clinical course, childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the consequences and medical resources for treatment in order to provide not only optimal QOL for patients, but also to garner additional information with the intent of minimizing what at present are severe consequences of both the disease and its treatment.

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