Late Effects Case Presentation Exeter Sept 2008

Ms J W Diagnosed Acute Lymphoblastic Leukaemia 1981 aged 5 yrs Diagnosed Acute Lymphoblastic Leukaemia 1981 aged 5 yrs UKALL X protocol BCH UKALL X protocol BCH 18 Gy prophylactic cranial irradiation 18 Gy prophylactic cranial irradiation Chemotherapy but no anthracyclines Chemotherapy but no anthracyclines

Ms JW First presented late effects clinic 06/2005 First presented late effects clinic 06/2005 No complaints No complaints No hormone replacement No hormone replacement 4 months Post Partum 4 months Post Partum Offered anterior pituitary function test Offered anterior pituitary function test T4 TSH “normal” T4 TSH “normal”

Ms J W Need for testing?? Need for testing?? 58% have one or more medical problem 58% have one or more medical problem 32% have two or more medical problems 32% have two or more medical problems 41% have an endocrine disorder 41% have an endocrine disorder Stevens et al 1998 Eur J can 34:694 Stevens et al 1998 Eur J can 34:694

Ms J W Glucagon 1/07/2006 Glucagon 1/07/2006 Pk GH <1.0 mU/L Pk GH <1.0 mU/L Pk Cortisol 311 nmol/L Pk Cortisol 311 nmol/L IGF nmol/L (14-48) IGF nmol/L (14-48) Borderline TFT’s Borderline TFT’s Ft4 9.7pmol/L Ft4 9.7pmol/L TSH 4.98mU/L TSH 4.98mU/L AGHDA 19/25 AGHDA 19/25

Ms J W Started on T4 100mcgs Started on T4 100mcgs SST needed SST needed Short synacthen test performed Short synacthen test performed 0mins) 222 nmol/L 0mins) 222 nmol/L 30mins) 572nmol/L 30mins) 572nmol/L

Ms J W September 2006 September 2006 T4 duration 5 weeks T4 duration 5 weeks Reported increased energy levels Reported increased energy levels Weight decreased 3kgs Weight decreased 3kgs “Feeling great” “Feeling great” AGHDA 21/25 AGHDA 21/25

Ms J W Glucagon 16/02/2007 Glucagon 16/02/2007 Pk Cortisol 286 nmol/L Pk Cortisol 286 nmol/L Pk GH 5.5 mIU/L (baseline) Pk GH 5.5 mIU/L (baseline) C/o profound lethargy C/o profound lethargy Started on hydrocortisone daily dose 10mgs/5mgs Started on hydrocortisone daily dose 10mgs/5mgs

Ms J W June 2007 June 2007 c/o profound fatigue c/o profound fatigue Headaches normally afternoons Headaches normally afternoons Ringing inner ears Ringing inner ears IGF1 increased to 51nmol/L (13-37) IGF1 increased to 51nmol/L (13-37) CT head large atypical looking meningioma CT head large atypical looking meningioma

MR

MR

MR

Ms J W Craniotomy and resection of tumour January 2008 Neuropathology meningioma WHO grade 1 Radiotherapy referral

Ms J W March 2008 March 2008 Extremely well Extremely well No headaches No headaches See September with follow up scans See September with follow up scans Continues hydrocortisone 10/5mgs Continues hydrocortisone 10/5mgs T4 100mcgs T4 100mcgs Irregular periods Irregular periods Sex steroids Sex steroids

Ms J W Summary Late Effects!! Late Effects!! Secondary tumours following prophylactic cranial irradiation Secondary tumours following prophylactic cranial irradiation Progressive hypopituitarism rarely observed at lose radiation dose Progressive hypopituitarism rarely observed at lose radiation dose Endocrinopathy Endocrinopathy 2 failed glucagons V normal/high IGF1 2 failed glucagons V normal/high IGF1 Normal SST Normal SST Borderline AST Borderline AST