Abstract
Craniopharyngioma as a rare tumor originating from cells of rathke’s
pouch and representing 2–5% of all intracranial tumors is a rare
and generally benign neoplasm of the central nervous system with two incidence
peaks one in childhood and one after 40 years of age. Data on adult patients is
scarce compared to childhood onset tumors, however the burden of disease caused
by the tumors and related treatment options is significant. Clinical symptoms
range from headaches, visual disability, cranial nerve affection or hypothalamic
symptoms (e. g. morbid obesity) to endocrine disorders. Most symptoms
are related to tumor mass effect. The current standard of diagnostics is the
determination of serum hormone levels and contrast enhanced magnetic resonance
imaging often resulting in surgical treatment which holds a key role in all
treatment concepts and should follow a hypothalamus sparing path. Radiation
therapy may prove beneficial as adjuvant therapeutic option or in recurrent
tumor, especially papillary tumors may be targeted using BRAF-600 inhibitors,
targeted therapies for adamantinomatous craniopharyngioma have not yet reached a
stage of clinical testing. Although prognosis regarding overall survival is
favorable, life expectancy may be reduced due to the tumor itself as well as due
to treatment effects. An important aspect especially in the adult population is
the reduction in quality of life which is comparable to primary malignant brain
tumors and metastases, calling for individual patient specific treatment
approaches.
Key words
Hypopituitarism - Insufficiencies - Cancer - Craniopharyngioma - QoL
