Craniopharyngioma: 12-years experience and results in a reference center

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 327-337-Neuroendocrine Tumors
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-328
Eva María Venegas Moreno*, Antonio Jesús Martínez Ortega, María Elena Dios Fuentes, Emilio José García García, Alberto Aliaga Verdugo, María Natividad García Hernández, Francisco Javier Márquez Rivas, Juan Manuel Polaina Bailón, Beatriz González Aguilera, Alfonso Leal Cerro and Alfonso Manuel Soto Moreno
Hospital Universitario Virgen del Rocío, Seville, Spain
Craniopharyngiomas  (CP) are locally aggressive tumors with benign histology derived from embryonic remnants of the craniopharyngeal duct, with an incidence in Spain of 0.5-2 cases/million inhabitants/year, and an estimated prevalence of 1-3/100000 inhabitants.  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 in our center between years 2000 and 2012.

Material and Methods: Descriptive retrospective study. We performed a systematic search in our clinical database between years 2000 and 2012, including all patients with postoperative diagnosis of CP. Total sample: n=58.

Results: Our sample is composed by n=20 (54.2%) children, n=38 (45.8%) adults; n=30 males (51.7% of total sample, 62.5% children, 50% adults);  median of age at diagnosis 7 years ( children), 43 yr (adults). Main neurological manifestations at diagnosis were visual impairment (66.7% children, 75% adults) and headache (50% children, 70.6% adults), as well as intracranial hypertension (50% children, 33.3% adults);  the most frequent endocrine abnormalities were diabetes insipidus (50% children, 16.7% adults), menstrual disorders and growth impairment. The preferred surgical approach was an open technique. Postoperative sequelae were present in 97.9% of total patients (100% children, 96.7% adults), usually 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). In 47.7% of cases, external adjuvant treatment was applied, mainly radiotherapy (100% conventional radiotherapy in children, while in adults 67% was stereotactic vs. 33% conventional). The disease relapsed in 46.7% of total patients (69.2% children, 37.9% adults) requiring re-intervention approximately 2 years after initial surgery. Mortality rate is up to 20% (n=12).

Conclusions: Our results are consistent with currently available 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 devastating consequences of both the disease and its treatment.

Nothing to Disclose: EMV, AJM, MED, EJG, AA, MNG, FJM, JMP, BG, AL, AMS

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm