Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review.
Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review.
复制标题
肾门异位肾上腺皮质腺瘤：病例报告及文献综述。
DOI：
10.1186/s13000-016-0490-6
复制
发表时间：
2016-04-19
影响因子：
2.6
通讯作者：
Wang EH
中科院分区：
文献类型：
--
作者：
Liu Y;Jiang YF;Wang YL;Cao HY;Wang L;Xu HT;Li QC;Qiu XS;Wang EH
异位（副）肾上腺皮质组织，也称为肾上腺残余，是肾上腺发育异常。最常见的异位部位靠近肾上腺，沿着性腺的下降或迁移路径，因为在胚胎发生过程中肾上腺皮质原基和性腺胚基之间的空间关系密切。异位残余物可能发生明显的增生，偶尔诱发异位肾上腺皮质腺瘤或癌。一名27岁的中国女性患者，闭经3个月，在超声检查发现左肾门有一个孤立的肿块后进行了计算机断层摄影尿路造影。组织学上，突出的嗜酸性肿瘤细胞形成了肺泡或腺泡样结构。免疫组化结果（α-肾上腺素+，Melan-A+，突触素+）显示肿瘤起源于肾上腺皮质，诊断为异位肾上腺皮质腺瘤。术后3个月随访，患者存活，无肿瘤复发或转移。异位肾上腺皮质腺瘤的特殊组织学表现，可能导致误诊为嗜酸细胞瘤或肾透明细胞癌，尤其是当标本有限时。这个病例提醒病理学家注意这种良性肿瘤的非典型病例。虽然不常见，异位肾上腺病变应包括在鉴别诊断的肿瘤涉及肾门。将这种良性疾病误诊为恶性肾肿瘤可能会对患者造成严重后果，包括不必要的根治性肾切除术。术前活检和适当的免疫组化染色将有助于确定肿瘤的起源和性质，并避免术中的不确定性。
Ectopic (accessory) adrenocortical tissue, also known as adrenal rests, is a developmental abnormality of the adrenal gland. The most common ectopic site is in close proximity to the adrenal glands and along the path of descent or migration of the gonads because of the close spatial relationship between the adrenocortical primordium and gonadal blastema during embryogenesis. Ectopic rests may undergo marked hyperplasia, and occasionally induce ectopic adrenocortical adenomas or carcinomas. A 27-year-old Chinese female patient who presented with amenorrhea of 3 months duration underwent computed tomography urography after ultrasound revealed a solitary mass in the left renal hilum. Histologically, the prominent eosinophilic tumor cells formed an alveolar- or acinar-like configuration. The immunohistochemical profile (alpha-inhibin+, Melan-A+, synaptophysin+) indicated the adrenocortical origin of the tumor, diagnosed as ectopic adrenocortical adenoma. The patient was alive with no tumor recurrence or metastasis at the 3-month follow-up examination. The unusual histological appearance of ectopic adrenocortical adenoma may result in its misdiagnosis as oncocytoma or clear cell renal cell carcinoma, especially if the specimen is limited. This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor. Although uncommon, an ectopic adrenal lesion should be included in the differential diagnosis of tumors involving the renal hilum. A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy. Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.