A man in his sixties with dyspnoea and oedema

A man in his sixties with chronic obstructive pulmonary disease was hospitalised due to oedema and dyspnoea during the previous weeks. He was hypertensive, with 10 kg weight gain, generalised oedema, proximal myopathy and moon face. The assessment was consistent with ectopic ACTH-dependent Cushing's syndrome. A 15 mm lung tumour was detected on CT, with inconclusive cytological examination, and negative FDG/PET CT and octreotide scintigraphy. He developed necrotising pancreatitis and a duodenal perforation, which were surgically treated. His cortisol levels and Cushingoid appearance normalised after surgery, and it was concluded that his hypercortisolism was part of a physiological response. He remained clinically in habitual shape until two years later, when he again developed Cushingoid stigmata. A new octreotide scintigraphy was negative, but FDG/PET CT revealed increased FDG uptake in the lung lesion. Before a lung biopsy was performed, the patient developed necrotising pancreatitis. He was treated conservatively and died in respiratory failure. Autopsy revealed a NET in the lung and necrotising pancreatitis.

The case demonstrates diagnostic challenges in the assessment of ectopic ACTH-dependent cyclic Cushing's syndrome. Is also suggests that pancreatitis could be triggered by hypercortisolism.