摘要
目的探讨涎腺分泌癌(secretory carcinoma,SC)的临床病理特征和预后。方法回顾性分析10例SC的临床病理资料,进行组织化学染色和免疫组化En Vision法检测,采用荧光原位杂交(fluorescence in situ hybridization,FISH)检测ETV6-NTRK3融合基因。结果 10例SC中,男性8例,女性2例;发病年龄22~62岁,平均45岁。9例发生于腮腺,1例发生于颌下腺;首发症状均为局部无痛性肿块。眼观:肿瘤呈结节状和分叶状,多数与周围组织界限不清;肿物直径1~3.5 cm,平均1.8 cm;切面呈实性,其中1例伴囊性变,灰白、灰黄或灰红色。镜检:肿瘤组织呈推挤式浸润性生长,少数呈膨胀性生长;肿瘤细胞排列成微囊状、囊性乳头状和腺泡状,微囊和腺泡腔内可见均质红染的嗜酸性分泌物。免疫表型:10例CKpan均弥漫强阳性,9例S-100、Mammaglobin、vimentin和CK7均弥漫强阳性,CD117、Dog-1、p53、p63、SMA和GATA3等均阴性。组织化学染色:肿瘤细胞PAS阳性,黏液卡红阴性。10例中4例行ETV6-NTRK3融合基因检测,3例阳性。10例均获得随访,随访时间2个月~35年,除1例术后16年复发和转移死亡外,其余9例均健在。结论 SC是新近认识的罕见涎腺肿瘤,其恶性程度低,生长缓慢、预后良好。组织学上微囊状结构和嗜酸性分泌物的存在是SC的重要组织学特征,免疫组化标记S-100和Mammaglobin均呈弥漫强阳性,有助于其诊断和鉴别诊断。分子遗传学上,有ETV6-NTRK3融合基因可确诊该肿瘤。
Purpose To investigate the clinicopathological features and prognosis of salivary gland secretory carcinoma （SC）. Methods A retrospective study was performed including reviewing the clinical documents and pathological sections of 10 cases of SC. Immunohistochemical EnVision study and histochemical staining were performed in the 10 cases. Fluorescence in situ hybridization （FISH） was used to detect the ETV6-NTRK3 fusion gene. Results There were 8 men and 2 women included in this study. The mean age was 45 years （ranged from 22 to 62 years）. 9 cases occurred in the parotid and 1 case in submandibular gland. Local painless masses werecommon first symptoms. Tumor size ranged from 1 cm to 3.5 cm in maximum diameter （ average 1.8 cm） and the cut surface of most tumors was solid with dusty red or grayish yellow color, of which 1 case showed cystic degeneration. Histologically, the tumors usually pushed and were invasive to the adjacent tissues. Sometimes, the tumors showed expansive growth pattern. Tumor cells demonstrated microcystic, cystic papillary and alveolar patterns. Eosinophilic secretions could be observed in both microcysts and lumen of alveolus. Immunohistochemically, 10 cases revealed diffuse positivity of cytokeratin, and 9 cases were diffusely and strongly positive for S-100, Mammaglobin, vimentin and CKT, whereas all cases were negative for CDl17, Dog-1, p53, p63, SMA, and GATA3. The tumor cells were positive for PAS staining and negative for mucicarmine staining. The detec- tion ETV6-NTRK3 fusion gene was carried out in 4 cases by FISH analysis, among which 3 cases were positive. Follow-up data were available in the 10 patients （ranged from 2 months to 35 years） , among which 9 patients were alive, except for 1 patient died of tumor recurrence and metastasis 16 years after surgery. Conclusion SC is a newly recognized rare malignanttumor of salivary gland with a low-grade malignancy, slow growth pattern and favorable prognosis. The histological structures of microcysts and eosinophilic secretions are the crucial histological characteristics of SC. Diffusely strong positive expression of S- 100 and mammaglobin is helpful for the diagnosis and differential diagnosis of the tumor. The diagnosis of SC could be confirmed when ETV6-NTRK3 fusion gene could be identified.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2018年第1期22-27,共6页
Chinese Journal of Clinical and Experimental Pathology
基金
国家自然科学基金青年基金(81201654)
青岛市市南区科技局课题(2016-3-035-YY)
关键词
涎腺肿瘤
分泌癌
免疫组织化学
荧光原位杂交
预后
salivary gland neoplasm
secretory carcinoma
immunohistochemistry
fluorescence in situ hybridization
prognosis
