Background: Childhood IGCT are rare, malignant tumours of the pituitary stalk and pineal region, highly curable (>90%) by multimodal therapies. Neuroendocrine outcomes are thus important. Deficits increase over time but, without longitudinal studies, it remains unclear whether they are primarily disease or treatment related.
Objective and hypotheses: To determine, by longitudinal retrospective analysis in survivors, tumour- and treatment-related factors for neuroendocrine morbidity, including effects of anatomical tumour location and volumetric size.
Method: We searched germinoma in electronic document libraries from 01/01/1995 until 01/01/2015 at our split-site centre (UCLH/GOSH) and excluded cases where an IGCT was not confirmed by MRI and/or histopathology reports. We identified 30 survivors now aged 14.7 (826) years and 5.5 (0.214.7) years from diagnosis. Evolving endocrinopathies were analysed by Kaplan Meir statistics and correlated with radiological tumour location and mass (3D volumetric assessments (www.itksnap.org)) and treatment.
Results: Patients were diagnosed at median age of 9.52 (5.8314.57) years, pineal tumours presenting with shorter symptom duration (pineal vs pituitary:0.33 vs 0.67 years, P=0.10). 76.7% had presenting visual disturbance, 57.1% had two or more anterior pituitary deficits and 66.7% had central DI (CDI). Pineal disease was of smaller volume (pituitary vs pineal: 5.74 (0.9613.99) vs 0.97 (0.242.89) cm3 P=0.02). All (100%) pituitary cases eventually developed CDI (vs 0% pineal), GHD and one or more other anterior pituitary deficits, (vs 16.7% pineal). All seven surgical patients developed perioperative panhypopituitarism+CDI, but only 50% of irradiated patients, (neuraxial alone (CSI) vs ventricular (VI)+chemo), developed new anterior pituitary deficits.
Conclusion: Contrary to prevailing dogma, late evolving endocrinopathy is predicted by pituitary disease at diagnosis rather than imposed pituitary radiation and escalated by surgery. Thus substituting CSI with VI+chemo will not avoid this complication and may add peripheral toxicity (ototoxicity, nephrotoxicity, gonadotoxicity).
01 - 03 Oct 2015
European Society for Paediatric Endocrinology