原发性肾上腺皮质癌的诊治分析

目的总结和提高原发性肾上腺皮质癌的诊治水平。方法回顾分析16例肾上腺皮质癌患者的临床资料。根据其临床症状、内分泌功能测定、影像学特点做出诊断，手术治疗并随访。结果16例患者中，内分泌功能紊乱者8例，以库欣征、性征异常、醛固酮增多症为主。超声、CT、MRI测定肿瘤直径为4．8～19．5cm，平均7．8cm。3例有远处转移。行根治性切除术13例，侵及同侧肾脏者做肾和肾上腙切除术2例，肾上腺肿瘤并腔静脉癌栓切除2例，腔静脉部分切除1例。病理结果：Ⅰ其耳2例，Ⅱ期8例，Ⅲ期3例，Ⅳ期3例。随访3-62个月，手术2年以上的11例患者中有6例仍存活，但1例肺转移，1例骨转移；死亡5例，平均存活26个月。结论肾上腺皮质癌患者预后差。影像学检查结合临床症状是早期诊断的关键，根治性手术是惟一有效的治疗方法。     

成人肾上腺皮质癌的诊断和治疗(附16例报告)

目的：探讨成人肾上腺皮质癌的临床特点和治疗方法。方法：回顾性分析16例成人肾上腺皮质癌患者的临床资料，全部患者术前行B超、CT等影像学检查，10例接受内分泌激素检测，均首先行手术治疗，完整切除肿瘤13例，未能完整切除者3例。结果：术前13例明确诊断，3例末确诊。术后病理检查I期肿瘤3例，Ⅱ期9例，Ⅲ期4例。随访N一62个月，平均生存48个月；有5例至今生存，其中3例因术后转移而进展为Ⅳ期，行联合化疗(PDD加ADM加VN16)3—5周期，均末达到肿瘤缓解，术后平均生存17个月。3例末完全切除者术后放疗均末控，均因肿瘤扩散死亡，术后平均生存期为11个月。结论：成人肾上腺皮质癌恶性程度高，影像学检查是确诊的关键；早期发现并行根治性切除手术是目前提高其生存率的唯一有效方法。     

Pituitary choristoma composed of corticotrophs and adrenocortical cells in the sella turcica

A pituitary tumour composed of well-differentiated corticotrophs and adrenocortical cells is reported. Sections of the tumour revealed a mixture of small round cells with amphophilic or basophilic periodic acid-Schiff (PAS)-positive cytoplasm and large spherical and oval cells with abundant, granular, partly vacuolated PAS-negative cytoplasm. The small cells contained type 1 cytokeratin-positive microfilaments, numerous 250–500 nm endocrine-type secretory granules immunoreactive for adenocorticotropic hormone (ACTH) and -lipotropin. The large cells possessed ample cytoplasm filled with abundant vesicular smooth endoplasmic reticulum, numerous mitochondria possessing tubulovesicular cristae and frequent dense bodies. They lacked the features of pituitary endocrine cells or folliculostellate cells and were found to contain a panel of steroidogenic dehydrogenases and hydroxylases. The tumour was classified as a choristoma, in which two distinct cells types, corticotrophs and adrenocortical cells, were mixed. We suggest that, under continued ACTH stimulation, uncommitted stem cells may differentiate into adrenocortical cells. Alternatively, the presence of adrenocortical cells may be the result of heterotopia.     

ADRENOCORTICAL CARCINOMA IN INFANCY

A case of adrenocortical carcinoma associated with congenital heart defect in a 6-month-old Japanese girl is reported. A fist-sized tumor was incidentally noted in the right hypochondrium upon admission for cardiac surgery. No clinical endocrinopathy was evident in this case. The resected tumor was encapsulated with smooth surface and no invasion to adjacent tissues or organs was observed. Histologically, the tumor was composed of small cells with granular or clear cytoplasm, and occasional giant cells with single or multiple nuclei. By electron microscopy, the tumor cells showed various nuclear contours with distinct nucleoli and had a moderate amount of cytoplasm containing abundant rough endoplasmic reticulum and mitochondria with variable-sized electron-dense granules. Intercellular desmosome-llke junctions were observed in some tumor cells. Immunohistochemlcally, the tumor cells contained granules positive for estriol, progesterone and Cortisol. These morphological findings including electron microscopic features suggested that the tumor cells had a malignant character.     

Primary adrenocortical nodular dysplasia with Cusghing's syndrome and cardiac myxomas

Summary In a family with 4 children, 2 had slowly developing Cushing's syndrome and were adrenalectomized between the ages of 18 and 28 years. As in other cases with familial Cushing's syndrome, primary adrenocortical nodular dysplasia, the so-called adrenocortical adenomatosis, was demonstrated. The brother, now 39 years old, is in good health. The sister, however, died at the age of 36 years. Autopsy revealed a cardiac myxoma of the left atrium. A cardiac myxoma had been found incidentally at autopsy of these two patients' oldest brother, a boy who died at the age of 4 years. Both siblings with Cushing's syndrome presented additional Peutz-Jegher-like hyperpigmentation and myxomatous tumours of the skin. Identical observations of familial adrenocortical nodular dysplasia, Cushing's syndrome and cardiac myxomas have not been reported to date. However, the familial occurrence of cardiac myxomas and adrenocortical dysplasia in combination with bilateral large cell calcifying Sertoli cell tumours of the testis has been published recently.     

利用CT表现鉴别肾上腺隐匿型嗜铬细胞瘤和皮质腺瘤

目的探讨如何利用CT表现对肾上腺隐匿型嗜铬细胞瘤和肾上腺皮质腺瘤进行鉴别诊断。方法收集2010年1月至2020年1月期间中国医科大学附属第一医院收治的177例肾上腺肿瘤患者进行回顾性分析,对比各组患者之间的一般临床资料和CT表现。结果隐匿型嗜铬细胞瘤56例、皮质醇腺瘤32例、醛固酮腺瘤44例、无功能腺瘤45例,隐匿型嗜铬细胞瘤组在肿瘤侧别上与无功能腺瘤组差异有统计学意义,在肿瘤最大直径、平扫CT值、动脉期和延迟期增强CT值上均显著大于三组肾上腺腺瘤组。以肿瘤直径≥2.95 cm诊断隐匿型嗜铬细胞瘤,曲线下面积(AUC)为0.872,敏感度为87.5%,特异性为76.0%;当平扫CT值≥24.5 Hu时,AUC为0.929,敏感度为94.0%,特异性为82.5%;当动脉期增强CT值≥89.5 Hu时,AUC为0.886,敏感度为72.7%,特异性为90.6%;当延迟期增强CT值≥82.5 Hu时,AUC为0.937,敏感度为84.6%,特异性为95.3%;联合以上四个指标时,AUC为0.981,阈值为≥0.118,敏感度为100%,特异性为90.6%。结论以肿瘤直径2.95 cm、平扫CT值24.5 Hu、动脉期增强CT值89.5 Hu和延迟期增强CT值82.5 Hu为阈值对肾上腺隐匿型嗜铬细胞瘤有较好的鉴别诊断价值。     

抑制素/激活素与肾上腺皮质肿瘤

研究显示,组成抑制素/激活素的亚单位α、βA、βB在正常肾上腺皮质有表达,在肾上腺皮质肿瘤中亦有表达。抑制素α亚单位基因突变与肾上腺皮质肿瘤有关。对抑制素基因敲除小鼠的研究显示,激活素可能是肾上腺皮质肿瘤发生的抑制因子,故认为它们可能与肾上腺皮质肿瘤的发生有关。抑制素、激活素通过影响类固醇生成及细胞凋亡,与骨形态生成蛋白相互作用调节醛固酮的生成,从而参与肾上腺皮质肿瘤的发生。抑制素与Calretinin合用可鉴别肾上腺皮质肿瘤与嗜铬细胞瘤,抗抑制素α亚单位抗体也可鉴别肾上腺皮质肿瘤与肾细胞癌。