鼻腔鼻窦原发分泌性癌2例临床病理学观察

Sinonasal mammary analogue secretory carcinoma:a clinicopathological analysis of 2 cases

目的探讨鼻腔鼻窦原发分泌性癌(secretory carcinoma,SC)的临床病理特征、诊断与鉴别诊断。方法收集2例鼻腔鼻窦原发SC,采用PAS、DPAS染色、免疫组化En Vision法检测CK、vimentin、S-100蛋白、SOX10、Mamaglobin、Calponin、DOG1、p63等的表达,并应用FISH法检测ETV6基因断裂。结果镜下肿瘤呈腺泡状、管状、实性及微囊状生长。细胞形态一致、温和,核小至中等大小,有时可见小核仁。胞质丰富呈泡状,核分裂象罕见,可见嗜酸性、胶样分泌物。PAS染色结果示瘤细胞内未见酶原颗粒,胶样分泌物阳性。瘤细胞弥漫表达CK、vimentin、S-100蛋白、SOX10、PAS,Mamaglobin、Calponin及p63不表达。FISH检测显示ETV6基因断裂。手术局部切除后,分别随访41、53个月,均无瘤存活。结论鼻腔鼻窦原发性SC属于少见的低度恶性肿瘤,组织学形态与部分涎腺肿瘤相似,结合免疫组化染色及ETV6基因检测可明确诊断。

Purpose To investigate the clinicopathological features of sinonasal primary secretory carcinoma （SC） and its diagnosis, differential diagnosis. Methods Two cases of sinnasal SC were analyzed by light microscopy with immunohisto- chemical staining （EnVision） for CK, vimentin, S-100 protein, SOX10, PAS, DPAS, Mamaglobin, Calponin, DOG1, p63 and molecular detection of ETV6 gene break. Results Morphologically, SC revealed varying proportions of solid, tubular, acinar, microcystic, tubular growth patterns. All SC cases were cytological low grade with uniform cells, small- to medium-sizes nuclei, occasional small nucleoli, and abundant pink, bubbly cytoplasm. Mitotic figures were rarely encountered. Tumor cells secreted eosinophilic, colloid-like secretions that were PAS positive. There were no DPAS positive zymogen granules in cytoplasm. This tumor cells were CK, vimentin, S-100, SOX10, PAS positive and Mamaglobin, Calponin and p63 negative. The ETV6 gene rearrangement was confirmed in all eases by fluorescence in situ hybridization （FISH）. After excision, all two pa- tients were survival without tumor recurrence for 41 months and 53 months. Conclusion Sinonasal primary SC is a low grade malignant tumor. The histological features of SC are overlap with other salivary gland tumors. Immunohistoehemical analysis and FISH are useful for the diagnosis and differential diagnosis.