Poster Presentation The International Congress of Neuroendocrinology 2014

Craniopharyngioma: a single center experience from 2000 to 2012. (#333)

Eva María Venegas Moreno 1 , Antonio Jesús Martínez Ortega 1 , Maria Elena Dios Fuentes 1 , Emilio José García García 2 , Alberto Aliaga Verdugo 1 , María Natividad García Hernández 1 , Francisco Javier Márquez Rivas 3 , Juan Manuel Polaina Bailón 3 , Beatriz González Aguilera 1 , Alfonso Leal Cerro 1 , Alfonso Manuel Soto Moreno 1
  1. Endocrinology & Clinical Nutrition, Hospital Universitario Virgen del Rocío, Seville, Spain
  2. Pediatric Endocrinology, Hospital Universitario Virgen del Rocío, Seville, Spain
  3. Neurosurgery, Hospital Universitario Virgen del Rocío, Seville, Spain

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.