Craniopharyngiomas (CP) are rare, locally aggressive tumors derived from embryonic remnants of the craniopharyngeal duct, with an incidence in Spain of 0.5-2 cases/million inhabitants/year. Our hospital is a Reference Center in the treatment and follow-up of this disease for both children and adults.
Objective: To describe the main features of patients diagnosed with CP admitted to our center between years 2000-2012.
Material&Methods: Descriptive retrospective study. We performed a systematic search in our clinical database, including all patients with postoperative diagnosis of CP between years 2000-2012.
Results: Total sample: n=58; n=20 (54.2%) children, n=38 (45.8%) adults; n=30 males (62.5% children, 50% adults); age at diagnosis (Median) 7 years (children), 43 yr (adults). Main neurological manifestations at diagnosis: visual impairment (66.7% children, 75% adults), headache (50% children, 70.6% adults), intracranial hypertension (50% children, 33.3% adults); main endocrine abnormalities at diagnosis: diabetes insipidus (50% children, 16.7% adults), menstrual disorders and growth impairment. The preferred surgical approach was an open technique. Postoperative sequelae: 97.9% of total patients (100% children, 96.7% adults), mainly panhypopituitarism (92.3% children, 89.3% adults), diabetes insipidus (81.8% children, 76.9% adults) and obesity/polyphagia (71.4 % children, 40% adults), visual impairment (72.7% children, 63.6% adults), epilepsy (37.5% children, 25% adults), hydrocephalus (30% children, 30.8% adults), CSF leakage (45.5% children, 10.5% adults), and psychiatric disorders (80% children, 26.3% adults). 47.7% of total cases required external adjuvant treatment, mainly radiotherapy (100% conventional radiotherapy in children; 67% stereotactic vs. 33% conventional in adults). Disease relapse rate: 46.7% (69.2% children, 37.9% adults) approximately 2 years after initial surgery. Mortality rate: 20% (n=12).Conclusions: Our results are consistent with previous evidence about CP, as we observe high rates of morbidity and mortality. These findings support the need of a multidisciplinary approach to this disease in order to minimize the consequences of both CP and its treatment.