肾上腺皮质癌临床病理特征及其网状纤维染色的意义

Clinicopathologic characteristics and reticular fiber staining of adrenocortical carcinoma

目的探讨肾上腺皮质癌(adrenocortical carcinoma,ACC)的临床病理特征及其网状纤维染色意义。方法收集20例肾上腺皮质腺瘤(adrenocortical adenoma,ACA)和16例ACC,分析两者的临床、影像学、病理特点、网状纤维染色,并复习相关文献。结果 ACA中位病程21个月,肿瘤最大中位直径2.6 cm;ACC中位病程5个月,肿瘤最大中位直径9.5 cm,9例诊断时已发生远处转移。ACA瘤细胞呈巢团状、粗梁状排列,胞质嗜酸或透明,核异型不明显,核分裂象罕见,无包膜、窦隙、静脉侵犯,出血、坏死、粗大胶原纤维少见。Weiss积分均≤2分,平均(0.85±0.81)分。ACC癌细胞呈巢团状、粗梁状、条索状或弥漫性排列,多数胞质嗜酸性,可见不同程度的核异型以及非典型核分裂,可见包膜、窦隙、静脉侵犯,出血、坏死、粗大胶原纤维易见。Weiss积分均≥3分,平均(5.2±1.3)分。免疫表型:ACA、ACC中vimentin、Syn、NSE、CK、CR、Melan-A、α-inhibin的表达比较差异无统计学意义(P>0.05)。网状纤维染色:20例ACA网状纤维结构规则完整,未见断裂、塌陷、稀疏或消失;16例ACC网状纤维结构破坏,不同程度的断裂、塌陷、稀疏或消失,两者网状纤维结构破坏率比较差异有统计学意义(P<0.01)。结论ACC的免疫表型缺乏特征性,直径增大、远处转移、粗大胶原纤维、网状纤维结构破坏对判断恶性特征具有重要意义。

Purpose To analyse the clinicopathologic char- acteristics and reticular fiber staining of adrenocortical carcinoma （ACC）. Methods The clinical, imaging, pathological data and reticular fiber staining of 20 cases with adrenocortical adenoma （ACA） and 16 cases with ACC were analyzed retrospective- ly. Reslflts The median course of disease and maximum diameter of ACA were 21 months and 2. 6 cm respectively, but ACC were 5 months and 9. 5 cm respectively. 9 cases with ACC were diagnosed with distant metastasis. Histologically, ACA cells arranged in nest-like and coarse trabecular forms with aci- dophilic or clear cytoplasm, unconspicuous nuclear atypia, and rare mitotic figures. The capsules, sinusoids and veins were un- involved, and hemorrhage, necrosis and thick collagen fibers were infrequent. The Weiss score of all ACA was less than 2 （average = 0. 85 ± 0.81 ）. ACC cells arranged in nest-like, coarse trabecular, cord-like, and diffuse structure with acidophilic cytoplasm, various nuclear atypia and atypical mitotic fig-ures. The invasion of capsules, sinusoids and veins, hemorrhage and necrosis regions and thick collagen fibers were easily found. The Weiss score of all ACC was more than 3 （ average = 5.2 ± 1.3 ）. Immunohistochemical staining showed that vimentin, Syn, NSE, CK, CR, Melan-A and α-inhibin were expressed similarly between ACA and carcinoma （ P 〉 0.05 ）. Reticular fi- ber staining revealed that the reticular fiber structures of ACA were unbroken, but ACC were destructed with various degrees of rupture, collapse, sparsity or disappearance （P 〈 0. 01 ）. Conclusion ACC lacks characteristic immune phenotype, but increased diameter, distant metastasis, thick collagen fibers and destructed reticular fibrillar network are helpful in the diagnosis of ACC.