肾上腺皮质疾病的临床病理学特点分析

Ciinicopathologic analysis of adrenocortical diseases

目的探讨肾上腺皮质疾病及肾上腺皮质球状带、束状带和网状带增生、腺瘤、腺癌的临床病理学特点及相互关系。方法天津医科大学总医院病理科1993—2008年外科病理检查总数167702例，其中肾上腺疾病910例（0．54％）、肾上腺皮质疾病631例（0．38％）。对检出率、构成比、平均诊断年龄、性别比例及临床表现、病理变化进行统计学分析。结果肾上腺皮质球状带、束状带、网状带病变分别为310例（49．13％）、319例（50．55％）、2例（0．32％）。16年中肾上腺疾病、肾上腺皮质疾病检出率和肾上腺皮质球、束状带疾病构成比及各带增生、腺瘤、腺癌的构成比均无变化趋势，均表现为腺瘤〉增生〉腺癌的发病规律。球、束、网状带功能性病变（90．97％、79．00％、100．00％）明显高于无功能性病变（9．03％、21．00％、0％），并均以女性多见。无功能性球状带腺瘤、腺癌和束状带增生、腺瘤的平均发病年龄分别高于功能性病变。球状带腺癌的发病年龄（62．7岁）明显高于增生（47．8岁）、腺瘤（44．8岁）；束状带腺癌平均年龄和男性平均年龄（44．3岁，50．0岁）分别高于腺瘤（44．0岁，49．1岁）、增生（41．5岁，40．9岁），而女性平均年龄（38．7岁）明显低于腺瘤（42．4岁）、增生（41．9岁），无功能性腺癌发病年龄最低（34．4岁）。功能性和无功能性腺癌直径均较大（球状带为4．3cm和4．0cm，束状带为7．1cm和8．0cm），癌细胞异形明显并伴较多出血、坏死和包膜、脉管侵犯。功能性肾上腺皮质球、束、网状带的增生、腺瘤中临床Conn、库欣或生殖器综合征伴有高血压症状比例明显高于不伴有高血压组或仅有单纯高血压症状组，但病变体积没有明显差异。其中功能性球、束状带腺瘤略小于无功能性，直径分别为1．7、2．3和2．3、2．4cm，球状带腺瘤平均直径小于束状带。查体发现的无功能性球、束状带腺瘤的直径大于非内分泌症状者（2．5、2．0和2．5、2．3cm）。伴生殖器综合征患者7例（男2例，女5例），其中束状带腺瘤4例、腺癌1例，网状带增生、腺瘤各1例。结论遗传学改变在肾上腺皮质疾病发生中具有重要作用，而环境因素对其发病趋势变化的影响相对较弱。影像学检查已作为。爵上腺皮质疾病的首选定位检查方法，对评价肿瘤良、恶性有一定提示作用。肾上腺皮质疾病临床表现与病理变化密切相关，确切的病变类型和肿瘤性质仍要由病理学检查确定。

Objective To investigate the correlationship between clinical and histopathological resuits of adrenocortical hyperplasia, adenoma and adenocarcinoma in the adrenocortical glomerular, fascicular and reticular zones. Methods A total number of 167 702 surgical specimens were collected by the Department of pathology at the General Hospital of Tianjin Medical University from 1993 -2008. Of these, there were 631 adrenocortical specimens. Statistic analysis was done with Run test, t or t＇test and ~2 test on data in the aspects of proportion rate, mean age, sex proportion and relationship between clinical and patho- logical results of adrenocortical diseases. Results The total number of adrenal diseases and adrenocortical diseases were 910 cases （0.54%） and 631 cases （0.38%） , respectively. The number of adrenocortical glomerular, fascicular, reticular diseases were 310 cases （49.13%） , 319 cases （50.55%） and 2 cases （0.32%） , respectively. The rate of adrenal diseases and adrenocortical diseases specimens did not change during the 16 year study period. The trend in proportion of hyperplasia, adenoma, adenocarcinoma in adrenocortical glomerular, fascicular and reticular zones did not change as well. The proportion pattern showed that there were more cases of adenoma than of hyperplasia and adenocarcinoma. The functional diseases of adrenocortical glomerular, fascicular and reticular zone （90.97% , 79.00% , 100.00% ） were higher than nonfunctional diseases in the corresponding zones （9.03% , 21.00% , 0% ） and both were higher in female patients. The mean age of nonfunctional patients with adenoma, glomerular zone adenocarcinoma and hyperplasia, adenoma of fascicular zone was higher than that in functional diseases. Interestingly, the mean age of glomerular zone adenocarcinoma patients （62.7 yrs） was higher than that in hyperplasia patients （47.8 yrs） and in adenoma patients （44.8 yrs）. The mean and male diagnostic ages of adenocarcinoma of fascicular （44.3 yrs, 50.0 yrs） were higher than that in adenoma （44.0 yrs, 49.1 yrs） and hyperplasia （41.5 yrs, 40.9 yrs） respectively. But the female mean diagnostic age of fascicular adenocarcinoma （38.7 yrs） was lower than that in adenoma （42.4 yrs） and hyperplasia （41.9 yrs） respectively and the lowest average age was for nonfunctional adenocarcinoma （34.4 yrs）. The diameters of the functional and nonfunctional adenocarcinoma were large （4.3 cm and 4.0 cm in glomerular zone, 7.1 cm and 8.0 cm in fascicular zone）. The carcinoma cells were in serious atyrpia, haemorrhage, necrosis and capsular or vascular infiltration. The rates of functional hyperplasia, adenoma with hypertension in Conn, Cushing or adrenogenital syndrome of adrenocortical glomerular, fascicular, reticular zone were higher than that without hypertension, hut their tumor diameters were similar. The diameters of functional adenoma in glomerular or fascicularer （ 1.7 cm, 2.3 cm ） were lower than that of nonfunctional adenoma （2.3 cm, 2.4 cm ） and the diameters of glomernlar tumors were smaller than that of fascicular tumors. Nonfunctional adenoma （2.5 cm, 2.0 cm ） of glo- merular or fascicular by check up finding were bigger than that of non-endocrine adenoma （2.5 cm, 2.3 cm）. There were seven cases with adrenogenital syndrome （2 male cases, 5 female cases） , among these, there were 4 fascicularer adenoma cases, 1 adenocarcinoma case, 1 network hyperplasia case and 1 adenoma Conclusions Genetic abnormality plays an important role in pathogenesis of adrenocortical disease and environment had less influence. Radiological examinations （ CT and MRI） of adrenocortical diseases are the first choice and have important roles in evaluating tumor characteristics. There is close relationship between clinical manifestation and pathology. However, the exact diagnosis of definite type of disease and nature of tumor needs to be made by pathologic examination.