【 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
【 预 览 】
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RO202303290004468ZK.pdf | 152KB | download |