BACKGROUND: Childhood ICGT 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 tumour location and volume. METHOD: We searched in electronic document libraries at our split-site centre (UCLH/GOSH) from 01/1995 until 01/2015 and excluded cases where an IGCT was not confirmed by MRI and/or histopathology reports. Endocrinopathies over time were analysed by Kaplan Meir statistics and correlated with tumour location and 3D volume using novel software- ITK-SNAPv3.2.0 (www.itksnap.org), and treatment type. RESULTS: 30 patients were identified and diagnosed at median age of 9.52 (5.83-14.57) years, pineal tumours presenting with shorter symptom duration (pineal vs pituitary:0.33 vs 0.67 years, p = 0.101). 76.7% had presenting visual disturbance, 57.1% had 2 or more anterior pituitary deficits and 66.7% had central DI. All (100%) pituitary cases eventually developed CDI (vs 0% pineal) and 2 or more anterior pituitary deficits, including 100% GHd (vs 16.7% pineal). All surgical patients developed panhypopituitarism + CDI, but only 50% of irradiated patients, (neuraxial(CSI) or ventricular (VI) + chemotherapy), 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).
. 2016 May 30;18(Suppl 3):iii45. doi: 10.1093/neuonc/now072.14
GC-14: ENDOCRINOPATHY AFTER INTRACRANIAL GERM CELL TUMOURS (IGCT) IS DISEASE NOT RADIATION- RELATED: TWO DECADES OF SURVEILLANCE IN A LARGE TERTIARY PAEDIATRIC COHORT
Eftychia Ioanna Dimitrakopoulou
1,2, Jasmine Chow
1,2, Joana Serra Caetano
1,2, Ash Ederies
1, Sara Stoneham
2, Helen Spoudeas
1,2
Eftychia Ioanna Dimitrakopoulou
1Great Ormond Street Hospital, London, UK
2University College London Hospital, London, UK
Find articles by Eftychia Ioanna Dimitrakopoulou
Jasmine Chow
1Great Ormond Street Hospital, London, UK
2University College London Hospital, London, UK
Find articles by Jasmine Chow
Joana Serra Caetano
1Great Ormond Street Hospital, London, UK
2University College London Hospital, London, UK
Find articles by Joana Serra Caetano
Helen Spoudeas
1Great Ormond Street Hospital, London, UK
2University College London Hospital, London, UK
Find articles by Helen Spoudeas
1Great Ormond Street Hospital, London, UK
2University College London Hospital, London, UK
Issue date 2016 Jun.
© the author(s) 2016. published by oxford university press on behalf of the society for neuro-oncology. all rights reserved. for permissions, please e-mail: journals.permissions@oup.com
PMCID: PMC4903344
