Endocrine Abstracts

Case history: Our patient is a 64 year old lady who had bilateral adrenalectomy in 1978 for Cushing”s disease. This was followed by radiotherapy in late 1978. To get further reduction of the ACTH producing pituitary adenoma she had transsphenoidal surgery in 1979 and then transfrontal craniotomy in 1982. This was followed by further radiotherapy in 1985. It left her with panhypopituitarism and she was on full hormone replacement. She presented to our hospital in late 2008 complaining of hyperpigmentation.

Investigations: Her ACTH was 5019ng/l (normalvalue 10-50) in 2005. In 2009 her ACTH had increased to 12468ng/l. Her MRI pituitary then showed bilateral parasellar lesions arising from the floor of the pituitary fossa and soft tissue lesions in both cavernous sinuses. In 2010 her ACTH levels were 34000-38000 and her hyperpigmentation was worse. In December 2010, she developed severe headache and MRI showed pituitary apoplexy.

Results and treatment: She was started somatostatin analogues and later established on Paseriotide 60 mg IM monthly. Further MRIs showed haemorrhagic component had resolved and in Dec 2012, there was some regression of the parasellar masses in the MRI of 2012. Her ACTH had dropped down to 5000ng/l. Presently her ACTH levels are 1398ng/l. Her pigmentation is much less and MRIs are showing stable masses. As a side effect of Paseriotide, she developed diabetes mellitus, which is well controlled on Metformin.

Conclusions and points for discussion: Nelson”s syndrome is a condition where there is enlargement of an ACTH producing tumour after bilateral adrenalectomy for the management of Cushing”s disease. The tumour continues to produce ACTH causing hyperpigmentation. We present a follow up case of a patient where long term treatment of over 10 years with Paseriotide has given good quality of life for a patient with Nelson”s syndrome. Pasireotide is a novel pituitary-directed somatostatin analogue with a high binding affinity to almost all somatostatin receptors.