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.     

肾上腺皮质腺癌的诊断与治疗

目的 探讨肾上腺皮质腺癌的早期诊断、手术及围手术期处理。方法 回顾分析了1990年至2001年间31例肾上腺皮质腺癌临床资料．并与1978年至1989年同种病例作比较。结果 近10年患者病理分期更趋向早期，更易行根治性切除，5年的存活率明显提高。结论 影像学的发展，使患者易早期诊断；围手术期的充分准备是减少手术并发症及死亡率的关键。     

原发性色素性结节状肾上腺皮质病(附4例报告)

目的：探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床表现和诊断治疗方法。方法：总结4例PPNAD的临床资料，4例均有库欣综合征的临床表现，内分泌检查结果提示为功能自主性肾上腺皮质肿瘤，但影像学检查并未发现肾上腺肿瘤。结果：4例患者均行单侧肾上腺全切除术，手术标本均表现为肾上腺大小正常或轻度增大，外表和切面上见黑色或深褐色小结节，结节间皮质萎缩，光镜下见组成结节的细胞体积大，脑质嗜伊红染色、颗粒状，部分细胞脑质中的颗粒状色素颗粒具脂褐质染色特征。结论：PPNAD在青少年中是一种引起库欣综合征的罕见病因，双侧肾上腺切除术是治愈本病的方法。     

自体吞噬与类固醇激素分泌调节的关系

本实验用腹腔内给予下丘脑激素或类固醇激素的方法，造成大鼠睾丸间质细胞和肾上腺皮质束状带细胞处于分泌兴奋或抑制状态，对这两种分泌类固醇激素细胞中的溶酶体和自噬小体进行了超微结构、细胞化学和形态计量研究。结果表明，在类固醇激素分泌增多时，细胞中自体吞噬活动减弱；激素分泌减少时，自体吞噬活动加强。这一结果证明了细胞内的自体吞噬活动与类固醇激素的分泌调节有密切关系，自体吞噬是溶酶体参与激素分泌调节过程的一种重要方式。     

Autoimmune polyendocrinopathy – candidosis – ectodermal dystrophy (APECED): autosomal recessive inheritance

A genetic analysis was made of 58 patients and their 42 families with APECED (autoimmune polyendocrinopathy--candidosis--ectodermal dystrophy). APECED is characterized by hypoparathyroidism, primary adrenocortical failure and chronic mucocutaneous candidosis, but none of its components is constant. Other endocrine deficiencies can occur as well and also dystrophy of dental enamel and nails. The proportion of affected siblings was 0.147 +/- 0.034 (S.D.) when corrected for truncate single ascertainment, 0.246 +/- 0.019 when corrected for a priori truncate complete ascertainment and 0.240 +/- 0.047 when corrected for a posteriori truncate complete ascertainment. The male/female ratio was 1.04. The results are compatible with autosomal recessive transmission. No heterozygous manifestations of the gene were found. The gene is enriched in isolated subpopulations in central and eastern Finland. APECED is part of the "Finnish heritage of disease".     

181例柯兴氏综合征的临床病理

本文收集自1957至1984年181例柯兴氏综合征的肾上腺标本,对30例作了电镜观察。对88例用电子计算机作逐步回归分析,结果表明治疗方式、病理类型、紫纹和性征异常对预后影响较大。并对肾上腺皮腺癌诊断标准的掌握、皮质增生中腺瘤样结节和皮质腺瘤间可能存在的转化关系、肾上腺皮质增生的诊断标准以及柯兴氏综合征病例肾上腺病损的超微结构进行了讨论。     

Non-functional adrenocortical adenoma with extensive degeneration

We report a case of non-functional adrenocortical adenoma of 5.5 x 5.5 x 3.2 cm in size that had an unusual histopathological appearance in two respects. First, the tumor contained small adipose foci with osteogenesis and was suspected of being a myelolipoma based on its appearance on computerized tomography (CT) and magnetic resonance imaging. However, pathologically, the fat element was seen focally and was not accompanied by hematopoietic cells, and the diagnosis of myelolipoma was abandoned. Second, the tumor was suspected of being an adrenal carcinoma based on its appearance on CT scans and showed extensive degeneration: fibrosis, hemorrhage, loss of parenchyma and moderate atypism of the tumor cells. However, as the architecture of the tumor cells was non-diffuse and there were no necrotic foci or mitoses, and vascular or capsular invasion were not present, the tumor was concluded to be an adrenocortical adenoma rather than a carcinoma. We diagnosed the tumor as a non-functional adrenocortical adenoma with extensive degeneration as the extensive areas of fibrosis were particularly remarkable. Furthermore, the extensive areas of degeneration might have been caused not only by an ischemic effect but also by low hormone levels.     

吸入性肾上腺皮质激素治疗哮喘和慢性阻塞性肺疾病

近年来,在支气管哮喘和慢性阻塞性肺疾病的治疗药物中,肾上腺皮质激素可对抗炎症或过敏介质,通过对慢性阻塞性肺疾病者的前炎症因子、炎症细胞、氧化应激的影响而发挥良好的疗效.吸入性肾上腺皮质激素疗效确切,对过敏介质选择性高,在治疗支气管哮喘、气管炎、慢性阻塞性肺疾病上显示了良好的前景.     

The Mother,The Psychoanalyst,The Poet and The Artist: Containment and Growth of The Mind

This paper considers the difficulties which humans have in using and developing their capacity for thought. Growth of the human mind, through attunement of the mother's unconscious experience with the infant's, is explored along with the distortions which arise because of aversion to uncertainty and suffering. It then looks at psychoanalysis and the verbal and visual arts in the light of the maternal function, and the way in which they also promote the growth of the mind. Four products of the human mind – an excerpt from an infant observation published in 1998, some post‐Kleinian psychoanalytic clinical case material from the 1980s, John Keats's 19th century Ode to a Nightingale and Lucas Cranach's 1526 painting, Adam and Eve – are used to illustrate some of the similarities and differences among them in giving conscious form to the unconscious.