Triple A syndrome: An unusual association with plurihormonal pituitary neuroendocrine tumour

Triple A syndrome is a rare genetic condition that can manifest in alacrima, achalasia, adrenal insufficiency, and commonly neurological disorders. We report on a patient with Triple A syndrome who underwent extensive workup for hyperhidrosis, subsequently found to have a pituitary neuroendocrine tumour causing acromegaly. Histopathology revealed an unusual plurihormonal PitNET of dual cell lineage. Previous studies have described tissue-specific expression of the AAAS gene in the cerebellum, pituitary gland, adrenal gland among other structures. This may explain the rare, reported disease phenotypes associated with Triple A syndrome and suggest need for early brain imaging.