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Diagnosis and Treatment > Signs and Symptoms

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Annika Sjoeholm Department of Women's and Children's Health, University of Otago, Dunedin School of Medicine, PO Box 56, Dunedin 9054, New Zealand

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Cassandra Li Department of Women's and Children's Health, University of Otago, Dunedin School of Medicine, PO Box 56, Dunedin 9054, New Zealand

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Chaey Leem Department of Women's and Children's Health, University of Otago, Dunedin School of Medicine, PO Box 56, Dunedin 9054, New Zealand

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Aiden Lee Department of Women's and Children's Health, University of Otago, Dunedin School of Medicine, PO Box 56, Dunedin 9054, New Zealand

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Maria P Stack Paediatric Nephrology, Starship Children's Hospital, Auckland, New Zealand

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Paul L Hofman Liggins Institute, University of Auckland, Auckland, New Zealand

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Benjamin J Wheeler 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

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Summary

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.

Learning points

  • 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.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Pituitary, Topic, Adrenal, Hormone, ACTH, Noradrenaline, Normetanephrine, Condition/ Syndrome, Adrenal insufficiency, Cushing's syndrome, Hypertension, Phaeochromocytoma, Patient Demographics, Age, Paediatric, Gender, Male, Ethnicity, Native Hawaiian/other Pacific Islander, Country of Treatment, New Zealand, Diagnosis and Treatment, Signs and Symptoms, Anxiety, Cutaneous pigmentation, Fatigue, Headache, Myasthaenia, Palpitations, Photophobia, Seizures, Sweating, Tremulousness, Vomiting, Investigation, ACTH, ACTH stimulation, Creatinine (serum), CT scan, Immunohistochemistry, MRI, Norepinephrine, Normetanephrine, Potassium, Ultrasound scan, Vanillylmandelic acid (24-hour urine), Intervention, Adrenalectomy, Medication, Alpha-blockers, Atenolol, Beta-blockers, Glucocorticoids, Labetalol, Phenoxybenzamine, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-15-0041
Volume/Issue:
Volume 2015: Issue 1
Open access