Endocrine Abstracts

A 45-year-old female developed clinical and biochemical recurrent Cushings’ Disease 2.5 years after transsphenoidal hypophysectomy for 2cm pituitary macroadenoma. Past medical history included Type 2 Diabetes mellitus, asthma, hypertension, grade 3 obesity, dyslipidaemia, depression and sleep apnoea. MRI showed residual pituitary tissue with left sphenoid sinus extension. MDT advised repeat transphenoidal surgery over radiotherapy due to presence of a clearly visible surgical target. Neurosurgery was initially delayed at patient request. She commenced metyrapone, titrated to cortisol levels but this was poorly tolerated. Whilst awaiting neurosurgery, she developed pituitary apoplexy with right sixth nerve palsy and superior quadrantanopia. MRI showed reduced tumor volume but she became progressively hypercortisolaemic. MDT discussion felt redo transphenoidal surgery no longer appropriate but unfortunately, the patient declined radiotherapy. Over the next 5 years, her metabolic control deteriorated, managed with intensive insulin regime, GLP-1 agonist and SGLT2 inhibitor, anti-hypertensives, statin, Levothyroxine for central hypothyroidism, prophylactic Rivaroxaban for thromboembolic risk and intermittent Metyrapone. Despite this she developed worsening alopecia, arthralgia, myopathy and NAFLD and became wheelchair dependent. Options of pituitary radiotherapy, bilateral surgical adrenalectomy or ablation were discussed. Bilateral surgical adrenalectomy was accepted by the patient but delayed due to Covid-19 pandemic. Whilst awaiting adrenalectomy she developed worsening headaches and superior quadrantanopia. Repeat MRI showed increase size of pituitary adenoma with supraseller extension. She commenced Pasireotide after MDT discussion but attended A/E with worsening headache, visual field defect and ongoing cushingoid symptoms after 3 doses. Repeat MRI scan showed no significant tumour growth or apoplexy and MDT recommended Osilodrostat, which the patient is awaiting.

Conclusion: This challenging case highlights the alternative options of managing resistant hypercortisolaemia in an individualized approach and the difficulty explaining complex treatment options with patients.