Endocrine Abstracts

A 59-year-old gentleman presented to the Medical Admission Unit with facial and ankle oedema following a dog bite. He did not have any significant past medical history. Initially, he was treated for angioedema. His oedema worsened to anasarca, blood pressure rose and was found to be hypokalaemic. Echocardiogram showed a normal left ventricular ejection fraction. Urine protein creatinine ratio was <0.5 g/24 h. Vasculitic and autoimmune screen were negative. Eight weeks later, he was noted to have a Cushingoid appearance, ongoing persistent hypertension with hypokalaemia, as well as a new diagnosis of diabetes mellitus. He had pulmonary oedema and ascites on clinical examination. He was treated with furosemide, spironolactone and insulin.

Subsequent biochemical investigations showed high random cortisol (2202 nmol/l) unsuppressed with low dose dexamethosone test, low ACTH (<5 ng/l) and high ACTH precursor (POMC) (610 pmol/l). He also had elevated fasting gut hormone levels – CART (469 pmol/l), chromogranin A (247 pmol/l), chromogranin B (216 pmol/l).

Imaging with MR and CT scanning showed liver metastases and bilateral adrenal nodules (largest measuring 27 mm). Further investigations showed these were somatostatin receptor negative. Liver biopsy demonstrated grade 3 poorly differentiated neuroendocrine cancer. PET/CT scan revealed high metabolic activity within the tail of pancreas, bilateral adrenal lesions and suspicious malignant liver lesions.

The patient underwent bilateral adrenalectomy, distal pancreatectomy and splenectomy. Pancreatic histology confirmed grade 3 poorly differentiated neuroendocrine cancer. Post operatively, his blood pressure, serum potassium and blood glucose have normalised.

At the local neuroendocrine multidisciplinary team meeting, chemotherapy has been recommended, and is awaiting assessment for the same.

Conclusion: This case denotes rapidly deteriorating ectopic Cushing’s syndrome following a misleading history of a dog bite which delayed diagnosis. It demonstrates the importance of correlating endocrine and biochemical findings with a clinical diagnosis.