Update on adrenal cortical neoplasia

The adrenal cortex gives rise to a biologically heterogenous group of neoplasms, each with a distinct morphology, antigen expression and molecular profile. Adrenal cortical adenomas have excellent prognosis and are usually cured by surgical resection alone, while adrenal cortical carcinomas are very aggressive tumors with a poor prognosis regardless of therapy. These tumors are rare and often challenging for a pathologist to diagnose, as significant overlap exists between benign and malignant lesions in some cases. In this review, we attempt to summarize most important histologic and clinical features of adrenal cortical adenomas and carcinomas, clarify the use of different grading systems, the use of special stains and the differential diagnosis for practicing pathologists. Most relevant hereditary syndromes associated with adrenal cortical tumors are listed. Updates in molecular alterations in adrenal cortical neoplasms and hyperplastic diseases as well as their clinical significance and potential therapeutic implications are also discussed.     

Disorders of sex development

Disorders of sex development (DSD) occur in 1–2/10,000 live births, with a specific molecular diagnosis only possible in 20% of cases. Presentation is usually at birth, and gender assignment must be avoided before review by an expert multidisciplinary team. Initial investigations allow a working diagnosis to be made within 48 hr. In 46,XY DSD, surgery may be necessary to correct hypospadias, reposition or remove undescended testes, and remove symptomatic Müllerian remnants. In 46,XX DSD, feminizing surgery is performed less frequently than in the past, but genitoplasty may still be indicated. Psychosocial support is required to promote positive adaptation as gender dissatisfaction can occur in certain conditions. Long-term outcome data are sparse.     

CT动态增强鉴别肾上腺乏脂肪腺瘤与肾上腺结节样增生的价值

目的探讨CT动态增强对鉴别肾上腺乏脂肪腺瘤与肾上腺结节样增生的价值。方法回顾性分析经手术病理证实的12例肾上腺乏脂肪腺瘤和27例肾上腺结节样增生患者的病例资料,统计两组病例各期增强CT值,利用公式灌注值P=CT静脉期-CT平扫期、廓清值C=CT静脉期-CT延迟期、绝对廓清灌注指数APC=(C/P)×100%及相对廓清灌注指数RAPC=(C/CT静脉期)×100%得到相关参数,并通过绘制受试者工作特征曲线,鉴别两者的最佳价值。结果肾上腺乏脂肪腺瘤直径较结节样增生更大;肾上腺乏脂肪腺瘤病灶各期CT值均明显高于结节样增生,且两者灌注值P、廓清值C、APC及RAPC有明显的统计学差异。年龄、性别、高血压、病灶位置、对侧肾上腺萎缩及动脉期CT值对鉴别两者无显著意义。结论CT动态增强扫描有助于鉴别肾上腺乏脂肪腺瘤和肾上腺结节样增生。     

Innervation of the zona fasciculata of the adult human adrenal cortex: a light and electron microscopic study

Summary Autonomic innervation of the adrenal cortex has been demonstrated in several species. Detailed ultrastructural studies on the innervation of the zona fasciculata of the normal human adrenal cortex are lacking. We report herein our observations on the pattern of innervation of the cells of the zona fasciculata of the normal adult human cortex at both the light and electron microscope levels. Postganglionic unmyelinated fibers were observed to descend from a dense capsular meshwork and to be distributed as delicate branches among the columns of endocrine cells. Immunoperoxidase staining confirmed the presence of nerve fibers in the zona fasciculata in a distribution similar to that observed after staining with silver impregnation methods. Ultrastructural findings lent further support to these observations by the demonstration of bundles of unmyelinated fibers with focal enlargements containing terminal boutons with both clear and dense core vesicles in close approximation with the endocrine cells.     

CT、MRI对肾上腺病变的诊断分析

目的:分析探讨CT、MRI诊断对肾上腺病变的作用与价值。方法:MarconiMX8000 4层螺旋CT机,增强造影剂使用非离子型制剂,常规扫描及增强。应用西门子MAGNETOM1.5T磁共振机,增强造影剂使用Gd-DTPA制剂,常规T1WI、T2WI、脂肪抑制、化学位移同反相位及增强。对25例经手术及病理资料诊断证实的肾上腺病变进行CT、MRI诊断回顾性分析。结果:可清楚显示肾上腺病变的侧别、数目、大小、范围及可能的性质。结论:CT是肾上腺病变最佳影像检查方法,MRI具有重要辅助及补充诊断价值。     

肾上腺区神经节细胞瘤(附18例报告)

目的较全面的了解神经节细胞瘤的临床特点及治疗方法。方法回顾性分析18例病理确诊的神经节细胞瘤患者临床表现、实验室检查、影像学检查和治疗效果。结果15例是无临床症状的影像学偶然发现病例,内分泌检查均在正常范围;另外3例以阵发性高血压为主要临床表现,其中1例尿儿茶酚胺升高,肾上腺髄质显像(MIBG)阳性。18例B型超声检查均为低回声;13例CT平扫见散在点状高密度影,12例CT增强扫描肿瘤周边强化明显。手术前诊断为嗜铬细胞瘤12例,神经节细胞瘤4例,肾上腺无功能腺瘤2例。18例行肿瘤切除,随访6个月~7年,高血压症状消失,2例肿瘤复发。结论肾上腺区神经节细胞瘤与肾上腺髄质肿瘤难以鉴别,综合各项影像学检查对诊断有较高的参考价值。手术治疗是唯一的有效方法。     

消化性溃疡的皮质醇分泌异常

采用放射免疫测定法研究了70例非应激性消化性溃疡患者的皮质醇分泌状态;并比较了患者与84例正常人的血清皮质醇浓度及其昼夜分泌节律。发现非应激性消化性溃疡血清皮质醇浓度明显高于健康对照组(P<0.01);然昼夜分泌节律相仿。结果提示,非应激性消化性溃疡有皮质醇分泌的明显异常。     

Adrenocortical responsiveness in neonates weaned from the ventilator with dexamethasone

Adrenocortical responsiveness was assessed in eight very low birthweight neonates who had bronchopulmonary dysplasia and had been weaned from mechanical ventilation using dexamethasone. Three of the eight infants did not respond to ACTH stimulation during the first week after cessation of dexamethasone, but all three responded normally when retested at least 1 month later. The present authors have thus demonstrated that some infants have at least temporary adrenocortical unresponsiveness after prolonged courses of glucocorticoid therapy, and suggest that adrenocortical function should be assessed in all infants who are weaned from mechanical ventilation using dexamethasone.