title: Adrenal insufficiency in a child following unilateral excision of a dual-hormone secreting phaeochromocytoma.
abstract: Phaeochromocytomas are a rare clinical entity, with dual hormone-secreting lesions particularly uncommon, seen in <1%. ACTH is the most common hormone co-produced, and is potentially lethal if not diagnosed. We present the case of a previously well 10-year-old boy, who presented acutely with a hypertensive crisis and was found to have a unilateral, non-syndromic phaeochromocytoma. Medical stabilization of his hypertension was challenging, and took 3 weeks to achieve, before proceeding to unilateral adrenalectomy. Post-operatively the child experienced severe fatigue and was subsequently confirmed to have adrenal insufficiency. He improved markedly with hydrocortisone replacement therapy, which is ongoing 6 months post-operatively. In retrospect this likely represents unrecognized, sub-clinical ACTH-dependent Cushing's syndrome secondary to an ACTH/or precursor dual-hormone secreting phaeochromocytoma. At follow-up, his hypertension had resolved, there was no biochemical evidence of recurrence of the phaeochromocytoma, and genetic analysis was indicative of a sporadic lesion.
  
  
  CONCLUSIONS
  Dual hormone secreting phaeochromocytomas with ACTH/or a precursor may cause secondary adrenal insufficiency following surgical removal.The concurrent features of Cushing's syndrome can be mild and easily overlooked presenting diagnostic and management pitfalls.As concomitant syndromes of hormone excess are rare in phaeochromocytomas; the diagnosis requires a high index of suspicion.Serial/diurnal cortisol levels, ACTH measurement +/- low dose dexamethasone suppression (when clinically stable, appropriate adrenergic blockade in place, and well supervised), can all be considered as needed.
authors.affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.
authors.forename: Annika
authors.initials: A
authors.lastname: Sjoeholm
authors.affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.
authors.forename: Cassandra
authors.initials: C
authors.lastname: Li
authors.affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.
authors.forename: Chaey
authors.initials: C
authors.lastname: Leem
authors.affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand.
authors.forename: Aiden
authors.initials: A
authors.lastname: Lee
authors.affiliation: Paediatric Nephrology, Starship Children's Hospital , Auckland , New Zealand.
authors.forename: Maria P
authors.initials: MP
authors.lastname: Stack
authors.affiliation: Liggins Institute, University of Auckland , Auckland , New Zealand.
authors.forename: Paul L
authors.initials: PL
authors.lastname: Hofman
authors.affiliation: Department of Women's and Children's Health, University of Otago , Dunedin School of Medicine, PO Box 56, Dunedin 9054 , New Zealand ; Paediatric Endocrinology, Southern District Health Board , Dunedin , New Zealand.
authors.forename: Benjamin J
authors.initials: BJ
authors.lastname: Wheeler