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.     

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。     

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.