Adrenal insufficiency in a child following unilateral excision of a dual-hormone secreting phaeochromocytoma

Endocrinology, Diabetes & Metabolism Case Reports

article

Annika Sjoeholm¹  Cassandra Li¹  Chaey Leem¹  Aiden Lee¹  Maria P Stack²  Paul L Hofman³  Benjamin J Wheeler¹ 

[1] Department of Women’s and Children’s Health, University of Otago, Dunedin School of Medicine;Paediatric Nephrology, Starship Children’s Hospital;Liggins Institute, University of Auckland;Paediatric Endocrinology, Southern District Health Board

关键词: Paediatric;    Male;    Native Hawaiian/other Pacific Islander;    New Zealand;    Adrenal;    Pituitary;    Adrenal;    Normetanephrine;    Noradrenaline;    ACTH;    Phaeochromocytoma;    Hypertension;    Cushing's syndrome;    Adrenal insufficiency;    Seizures;    Headache;    Photophobia;    Fatigue;    Anxiety;    Sweating;    Palpitations;    Vomiting;    Tremulousness;    Myasthaenia;    Cutaneous pigmentation;    CT scan;    MRI;    Ultrasound scan;    Creatinine (serum);    Potassium;    Normetanephrine;    Norepinephrine;    Vanillylmandelic acid (24-hour urine);    Immunohistochemistry;    ACTH;    ACTH stimulation;    Adrenalectomy;    Alpha-blockers;    Beta-blockers;    Labetalol;    Phenoxybenzamine;    Atenolol;    Glucocorticoids;    Unique/unexpected symptoms or presentations of a disease;    June;    2015;   

DOI  :  10.1530/EDM-15-0041

学科分类：血液学

来源: Bioscientifica Ltd.

【 摘 要 】

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 ofa 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 toachieve, before proceeding to unilateral adrenalectomy. Post-operatively the child experienced severe fatigue and wassubsequently 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-dependentCushing’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, andgenetic analysis was indicative of a sporadic lesion.

【 授权许可】

CC BY-NC-ND