An interesting case of acromegaly — ASN Events

An interesting case of acromegaly (#7)

Patrice Forner 1 , Ann McCormack 1
  1. St Vincent’s Hospital Sydney, Darlingurst, NSW, Australia

A 21-year-old female presented with a one-month history of chest pain and shortness of breath. She was found to have recurrence of a left atrial myxoma that was excised 5 years earlier. She underwent an excision of a 27 x 25mm atrial myxoma. On weaning sedation she had a right-sided motor deficit and gaze palsy. A CT-brain revealed a large MCA stroke. She underwent ECR, compressive hemicraniectomy and EVD placement.  A repeat CT-brain showed marked enlargement of the pituitary gland with extension into the sella turcica. An MRI brain confirmed the presence of a 19 x 27 x 20mm mass arising from the sella and extending superiorly into the suprasellar space. The patient had clinical features of acromegaly. Visual fields were intact. There was no family history of endocrinopathy or cardiac tumours. A pituitary panel revealed an elevated GH (23.3mU/L) and IGF-1 (66.9nmol/L), TSH was 0.15mIU/L, T4 6.6pmol/L, T3 8.8pmol/L. A short synacthen test was performed in the context of a relatively low early morning cortisol (245mmol/L). Baseline cortisol was 390nmol/L, ACTH 4.1pmol/L with a robust response to synacthen (600nmol/L and 665nmol/L at 30 and 60 minutes). A thyroid ultrasound revealed multiple TI-RADS-1 nodules. A CT abdomen revealed nodular adrenal glands, with a focal nodule of the left adrenal body. Radiological features were consistent with an adrenal adenoma. A 1mg dexamethasone suppression test failed to suppress with a morning cortisol of 274nmol/L. A 24-hour urinary free cortisol failed to confirm hypercortisolism with a cortisol concentration of 38nmol/L and cortisol excretion of 59nmol/d.  3-Methoxytyramine, plasma metanephrine and normetanephrine levels were within normal range. DHEAS was 1.1umol/L, oestradiol 81pmol/L, FSH 4.3IU/L, LH 3.7IU/L. Aldosterone/Renin was 0.8. She was commenced on Lanreotide 90mg monthly and thyroxine 50mcg daily for the management of central hypothyroidism. Her genetic profile is pending.

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