Endocrine Abstracts

A 42-year-old man presented to an outside hospital in 2015 with a 5-year history of increasing shoe size, ring size and low mood. During this 5-year period, he was also diagnosed with type 2 diabetes mellitus and obstructive sleep apnoea. On examination, he had signs consistent with acromegaly. This was confirmed on biochemical testing with an IGF-1>1200 mg/l, along with partial hypopituitarism and raised prolactin. His formal visual fields testing demonstrated a bilateral upper field defect. His MRI scan showed a large pituitary adenoma with compression of the optic chiasm. He was started on Cabergoline and hormone replacement with testosterone and levothyroxine. He was offered trans-sphenoidal surgery, which was complicated by postoperative CSF leak, meningitis and the development of seizures requiring anti-epileptic medications. Despite surgery, he had on-going symptoms and a persistently raised IGF-1 and he therefore underwent external beam fractionated radiotherapy. He was started on Lanreotide and up-titrated to maximum dose. 6 years after his initial diagnosis, although he had an improvement in his symptoms, he was still reporting symptoms of growth hormone excess and was particularly anxious to have corrective maxillofacial surgery to his jaw. He had a low energy state, stiffness and swelling in both of his hands and underwent bilateral carpal tunnel release surgery. He reported excessive sweating even with minimal exertion and had restless sleep with his sleep apnoea. He was referred to our centre. His IGF-1 remained raised at 272 mg/l (73.9-228.5 mg/l), although his growth hormone was in the safe range. He had a repeat MRI scan which showed a large predominantly residual tumour sitting close to the optic chiasm and stretching the right pre-chiasmatic optic nerve. The anatomy, together with the previous surgical complications, was thought unfavourable for further surgery. The tumour was too close to the optic nerves for stereotactic radiosurgery. The decision was then made to stop the Lanreotide and start on Pegvisomant. 2 years following starting Pegvisomant his latest IGF-1 is 216 mg/l. His symptoms have improved and he is now awaiting OMFS surgery. The primary treatment option for acromegaly is trans-sphenoidal surgery and can lead to complete remission of disease in 50% of patients with macroadenomas. Medical therapy options, in those not cured following surgery or not suitable for surgery, include growth hormone receptor antagonists, somatostatin receptor ligands and dopamine agonists. Radiotherapy can also be used in conjunction with the above to target inaccessible tumour and where medical therapy is not tolerated.