Cushing's Syndrome Due to an ACTH‐producing Neuroendocrine Tumour in the Nasal Roof

Abstract

A patient with ectopic adrenocorticotrophic hormone (ACTH) production from a neuroendocrine tumour of the nasal roof is presented. By indirect immunoperoxidase techniques the tumour cells were shown to be distinctly positive for ACTH and β‐endorphin but negative for other peptides derived from pro‐opiomelanocortin. Neither corticotropin releasing hormone (CRF) found in some tumours associated with ectopic Cushing's syndrome, nor gastrin immunoreactivity, which coexists with ACTH in normal rat pituitary and in rat and human gastrointestinal cells, were demonstrable in the tumour. A review of other, previously recognized locations of CRF/ACTH producing tumours is given to increase the awareness of the ectopic Cushing's syndrome, which may lack the classical features and is characterized by fulminant clinical course, extreme fatigue, weakness, pale facial swelling, oedema and hypokalaemic alkalosis.

Location of ectopic adrenocortical hormone‐secreting tumors causing Cushing's syndrome in the paranasal sinuses

“…He had decreased proximal muscle strength. Abnormal laboratory studies included white count 16,000/mm 3 (4000-10,000) with 93% neutrophils (36-75), serum potassium (2.2 [3.5-5]) and CO 2 (40 mOsm/L [22][23][24][25][26][27][28][29][30][31][32][33][34]), calcium 7 mg/ dL (8.6-10.3), phosphorus 1.8 mg/dL (2.7-4.6), parathyroid hormone (PTH) 153 pg/mL (12-75), 25-hydroxy vitamin D < 6 ng/mL , and serum albumin 2.4 g/dL (3.5-4.9). Radiographic examination of the chest revealed minimal left pleural effusion and faint nodular opacity over the anterior portion of the second left rib consistent with callus surrounding an old fracture as demonstrated in a subsequent CT scan.…”