Endocrine Abstracts

Background: Craniopharyngiomas (CPs) are epithelial tumors that typically arise in the suprasellar region. They are associated with high levels of morbidity related to tumour location and/or treatment-related injuries. Recent discoveries have also led to a better understanding of CP development and potential treatments.

Objetive: To analyse the clinical, pathological, molecular features and outcomes of patients with CP.

Design: Retrospective observational study.

Methods: Clinical records review and re-evaluation of histologic samples from patients who underwent surgery of CP in our institution between 1999 and 2016.

Results: There were 26 patients (53.8% male), mean age at diagnosis 37 years (range 2–73). Mean follow-up was 86 months (range 3–288). The commonest presenting symptoms were: visual alterations 84% (n=21), headaches 53.8% (n=14), and behaviour disorders 11.4% (n=3). 92.3% (24/25) of tumours had suprasellar involvement, with cystic component in 80.8% (16/26). Median tumour size was 30.4 mm (12–50). Initial surgical approach was transcranial in 96.2% (n=25). Available histological and molecular results were: 72% (n=18) adamantinomatous CP and 28% (n=7) papillary CP (PCP). Three PCP harboured BRAF V600E mutation. In these cases, the average number of surgical interventions were higher (4 vs1.3) and time to recurrence was shorter (34 vs 99 months). Three patients (11.4%) undergone radiation therapy. Last neuroimaging assessment showed residual tumour in 50% of patients. At the end of follow-up, panhypopituitarism and diabetes insipidus were detected in 72% (18/25) and 75% (18/24) respectively. Symptoms related to hypothalamic dysfunction were found in 76% (19/25). The mortality was 11.5% (n=3). Causes of death were: initial surgery related complications (1), multiple recurrent PCP harboured mutation BRAF v600E (1) and cardiovascular disease (1).

Conclusions: In our study, treatment of craniopharyngiomas were associated with high rates of tumor persistence and substantial morbidity. Tumours harboured BRAF V600E mutation seemed to be associated with poor prognosis.