Prolonged survival following late diagnosis and conservative management of pituitary apoplexy with severe hypopituitarism (#327)
A 53-year-old man with hereditary haemochromatosis requiring regular venesections was referred for assessment of hypogonadism, fortuitously just prior to undergoing elective TURP. His symptoms included fatigue, lethargy, proximal muscle weakness, reduced exercise capacity, poor libido, body hair loss, unintentional weight loss, myalgias and arthralgias. These symptoms developed acutely two years earlier, immediately following an extremely severe headache of 48 hours duration, which was not investigated. A previous trial of testosterone replacement had resulted in improved libido but overall symptomatic deterioration.
On examination he was pale and thin, with moderate proximal muscle weakness, very sparse body hair and a postural fall of >20 mmHg systolic; his thyroid gland was atrophic and his visual fields normal.
Initial pathology results confirmed hypopituitarism, with mid-afternoon serum cortisol <35 nmol/L, free T4 7.1 pmol/L, TSH 2.3 mU/L, free testosterone <0.5 pmol/L, FSH 3 U/L and LH 1 U/L. Additional investigations after commencement of glucocorticoid replacement included ACTH <5 ng/L, IGF-1 9 nmol/L, GH <0.5 mIU/L and PRL 88 mIU/L (RR<500). His serum ferritin was 886 mcg/L (RR 30-300) and his PSA was <0.1.
Review of previous pathology tests showed that he had been hypogonadal and hypothyroid 12-15 months earlier. Pituitary MRI showed a 10x8mm haemorrhagic mass consistent with an adenoma occupying the left side of the sella turcica, with displacement of normal pituitary tissue. There was no suprasellar extension and only mild pituitary stalk displacement.
A diagnosis of hypopituitarism secondary to apoplexy was made; his TURP was deferred. He was commenced sequentially on cortisone acetate, thyroxine and testosterone undecanoate in physiological doses, which resulted in marked and ongoing symptomatic improvement and an excellent quality of life.
This is the first case report of pituitary apoplexy in a patient with haemochromatosis. Pituitary apoplexy may be managed conservatively, if there is no visual disturbance, without increasing the risk of long-term hypopituitarism1. This patient’s severe glucocorticoid deficiency may well have been fatal if he had developed an acute illness or undergone elective surgery prior to diagnosis.
1Freda PU, Beckers AM, Katznelson L, Molitch ME, Montori VM, Post KD, Vance ML Journal of Clinical Endocrinology and Metabolism 2011; 96:894-904.