Endocrine Abstracts

A 33-year-old patient with background of Bilateral achilles tendonitis, was referred to Endocrinology with raised IGF-1 and concern of acromegaly. Her symptoms included arthralgias and change in hand and feet size. Pituitary profile confirmed elevated IGF-1 181 nmol/l, otherwise normal pituitary profile. On OGTT, there was failure to suppress Growth hormone. Pituitary MRI revealed large macroadenoma with suprasellar extension, extension into right cavernous sinus with optic chiasm compression and upward displacement. Surgery was advised, with initiation of adjuvant somatostatin analogue (lanreotide)therapy, but this caused gastrointestinal side-effects, needing addition of creon, which allowed her to continue lanreotide. Transsphenoidal surgery allowed significant debulking of disease with small residual left around carotid-artery. Histopathology revealed Somatotroph tumour with MIB1 index 10%. Genetics screen returned negative. Post op investigations unfortunately demonstrated persistent IGF-1 elevation 67 nmol/l and residual intrasellar tissue on MRI. It was advised to manage her medically in view of her wishes to complete her family, therefore lanreotide was restarted. Cabergoline was added at 0.5 mg once weekly but discontinued soon due to gastrointestinal side-effects. Lanreotide frequency was increased to 120 mg every 3 weeks. A year later, IGF-1 was mildly raised:32 nmol/l. She became pregnant leading to cessation of lanreotide but unfortunately had a miscarriage. She stayed off lanreotide and conceived again. She was monitored with visual-field assessment in each trimester. Pregnancy was uneventful apart from development of diet-controlled GDM. Post partum MRI revealed stable radiological disease and IGF-1 was 37 nmol/l. An 8 mm terminal ileum Neuroendocrine Tumour (NET) was identified on her routine colonoscopy. Polypectomy was not possible, so right hemicolectomy was undertaken to remove well-differentiated Grade1 NET Ki67<1% pT2pN1(1/16)LV1PN1R0. Lanreotide was reinitiated but optimal control of IGF-1 level wasn’t achieved. As her family was completed, she was re-reviewed in our pituitary clinic for definitive management options. Neurosurgeons felt that the small residual couldn’t be removed safely, hence she was referred for radiotherapy. Her lanreotide was switched to pegvisomant. She has received 45 Gy fractionated stereotactic radiotherapy and is awaiting a repeat MRI scan 6 months post-radiotherapy. We plan to follow her up in Endocrine clinic with annual pituitary profile and would reassess her pegvisomant dose in 6 months post-radiotherapy on basis of IGF-1 levels. Her last IGF-1 was finally in the normal range at 25.5 nmol/l.

Conclusion: This case elucidates how challenging it can be to manage acromegaly, especially in young patients who desire fertility and illustrates various treatment options available for management in acromegaly.