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目的 探讨胰高血糖素瘤 (glucagonoma ,GCGN)临床病理及其诊断与鉴别诊断。方法 对 4例GCGN进行光镜、免疫组化观察和其中 2例超微结构检测并结合文献进行分析。结果 GCGN以成人为主 ,女性稍多 ,早期临床无特征性 ,往往以皮疹、消瘦、贫血、糖尿病等某一现象为症候 ,血浆胰高血糖素 >5 0 0ng/L有诊断价值。影像学检查为实性或囊实性结节 ,肿瘤大小直径 1 5～ 14cm× 14 5cm× 16cm。病理形态表现为组织结构多样性 ,细胞形态多形性 ,免疫表型瘤细胞表达Glucagon ,Vimentin ,CgA ,Syn ,S - 10 0蛋白 ,超微结构胞质内富有不均一电子密度大小不同分泌颗粒。结论 GCGN患者临床表现、组织形态和超微结构无特征性 ,临床诊断和病理诊断有赖于免疫组化染色和血浆胰高血糖素检测 ,肿物 >5cm或瘤细胞表达PCNA和Ki6 7对判断肿瘤良恶性有意义。
Objective To investigate the clinical and pathological features of glucagonoma (GCGN) and their diagnostic and differential diagnosis. Methods Four cases of GCGN were observed by light microscopy, immunohistochemistry and two cases of ultrastructural examination combined with literature analysis. Results GCGN mainly in adults, slightly more women, early clinical features, often with rash, weight loss, anemia, diabetes and other symptoms as a symptom, plasma glucagon> 500ng / L have diagnostic value. Imaging examination was solid or cystic solid nodules, tumor size diameter of 15 ~ 14cm × 14 5cm × 16cm. The histopathology showed the diversity of tissue structure and the shape of cells. The immunophenotype tumor cells expressed Glucagon, Vimentin, CgA, Syn and S - 100 proteins. The ultrastructural cytoplasm was rich in heterogeneous electron density and secretory granules . Conclusion The clinical manifestation, histomorphology and ultrastructure of GCGN patients have no characteristic. The clinical diagnosis and pathological diagnosis depend on the immunohistochemical staining and plasma glucagon detection. The expression of PCNA and Ki67 in tumor> Benign and malignant meaningful.