National Institutes of Health (NIH), The Eunice Kennedy Shriver National Institute of Child Health andHuman Development (M.K.C., J.S., D.P.M.), Bethesda, Maryland 20892, USA.
J Clin Endocrinol Metab. 2012 Nov;97(11):E2084-9. doi: 10.1210/jc.2012-2298. Epub 2012 Aug 17.
Adrenalectomy is an experimental treatment option for select patients with congenital adrenal hyperplasia who have failed medical therapy. After adrenalectomy, adrenal rest tissue can remain in extraadrenal locations, cause recurrent hyperandrogenism, and be difficult to localize.
The aim of the study was to investigate the usefulness of positron emission tomography/computerized tomography (PET/CT) in identifying adrenal rest tissue.
A female with salt-wasting 21-hydroxylase deficiency who had bilateral adrenalectomy at age 17 yr presented with hyperandrogenism at age 32 yr. Pelvic magnetic resonance imaging and ultrasound imaging were nondiagnostic for the source of androgen production.
A baseline F-18 labeled fluoro-2-deoxy-d-glucose (18F-FDG) PET/CT scan showed no active uptake; however, a second scan preceded by a 250-μg cosyntropin injection identified three areas of active uptake near both ovaries. Subsequent ovarian venous sampling showed elevations in 17-hydroxyprogesterone, androstenedione, and 21-deoxycortisol in both ovarian veins compared to a peripheral vein at baseline and more so after cosyntropin administration. At laparoscopy, three well-circumscribed nodules (2.4 × 0.9 × 1.3 cm, 1.2 × 1.5 × 1.5 cm, and 2 × 1.5 × 1 cm) lying lateral to the fallopian tubes adjacent to the broad ligaments were removed. The paraovarian nodules and previously removed adrenal glands had similar histology and immunohistochemistry. Postoperatively, androgen concentrations were undetectable, with no response to cosyntropin stimulation.
Patients with CAH after an adrenalectomy may experience recurrent hyperandrogenism due to adrenal rest tissue. 18F-FDG PET/CT with cosyntropin stimulation accurately identified adrenal rest tissue not visualized with conventional imaging, allowing for successful surgical resection.
对于经药物治疗失败的特定先天性肾上腺增生患者，肾上腺切除术是一种实验性治疗选择。肾上腺切除术后，肾上腺残部组织可能会留在肾上腺外位置，导致复发性高雄激素血症，并难以定位。
本研究旨在探讨正电子发射断层扫描/计算机断层扫描（PET/CT）在识别肾上腺残部组织中的作用。
一名女性，17 岁时因盐皮质激素缺乏型 21-羟化酶缺陷行双侧肾上腺切除术，32 岁时出现高雄激素血症。盆腔磁共振成像和超声检查不能确定雄激素产生的来源。
基线 F-18 标记氟代-2-脱氧-d-葡萄糖（18F-FDG）PET/CT 扫描未见活性摄取；然而，在注射 250μg 促皮质素前进行的第二次扫描发现双侧卵巢附近有三个活性摄取区域。随后的卵巢静脉取样显示，在基线时和促皮质素给药后，双侧卵巢静脉中的 17-羟孕酮、雄烯二酮和 21-脱氧皮质醇均高于外周静脉。腹腔镜检查发现，三个边界清楚的结节（2.4×0.9×1.3cm、1.2×1.5×1.5cm 和 2×1.5×1cm）位于输卵管旁外侧靠近阔韧带，切除了这些结节。这些副卵巢结节和之前切除的肾上腺组织具有相似的组织学和免疫组织化学特征。术后，雄激素浓度无法检测到，且对促皮质素刺激无反应。
肾上腺切除术后的 CAH 患者可能因肾上腺残部组织而出现复发性高雄激素血症。18F-FDG PET/CT 联合促皮质素刺激能准确识别常规影像学无法显示的肾上腺残部组织，从而成功进行手术切除。
