涎腺分泌性癌10例临床病理学分析

Secretory carcinoma of salivary gland:a clinicopathologic analysis of ten cases

目的涎腺分泌性癌(secretory carcinoma of salivary gland,SCSG)具有独特的病理学特征,但部分组织学形态易与多种涎腺肿瘤混淆.本研究回顾性分析10例SCSG患者临床病理学特征,以期为其临床诊治提供参考.方法收集2007-01-01-2018-12-20临沂市肿瘤医院病理科存档的10例SCSG,观察其组织学形态、免疫组化及分子遗传学特点.结果 10例患者中发病年龄17~67岁,发生于左腮腺7例,右腮腺3例.临床表现均以无痛性肿块为首发症状,肿瘤最大径约1~3.5 cm.镜下瘤细胞以微囊状、管状、乳头状及实性生长为主,腔内可见嗜酸性或嗜碱性胶样/泡沫状分泌物,细胞核轻度异型.免疫组化结果显示,10例SCSG均弥漫表达CK7、S-100、Mammaglobin,其中8例局灶表达CK5/6;所有患者均不表达Dog-1、CD117、p63和calponin,Ki-67均<5%.10例SCSG标本均行荧光原位杂交技术(flu-orescence in situ hybridization,FISH)检测,其中8例具有特征性的ETV6-NTRK3融合基因,2例荧光信号太弱无法分析.结论大多数涎腺分泌性癌是一种低度恶性肿瘤,根据特征性的组织学形态及免疫组化即可明确诊断,在分子遗传学方面绝大多数患者有ETV6-NTRK3基因融合,但其具体分子分型还有待进一步研究.

OBJECTIVE Secretory carcinoma of salivary gland(SCSG) has unique pathological characteristics, but some histological morphologies are easily confused with many salivary gland tumors. This study retrospectively analyzed the clinicopathological characteristics of 10 cases of SCSG in order to provide reference for its clinical diagnosis and treatment. METHODS Ten cases of SCSG were collected from Linyi Tumor Hospital from January 1,2007 to December 20,2018. The histopathological,immunohistochemical and genetic features were analyzed. RESULTS Among these 10 cases, 7 cases occurred in the left parotid region and 3 cases occurred in the right parotid region. The age ranged from 17 to 67 year old. All the investigated SCSG patients were treated with painless mass as the first symptom. The maximum diameter of SCSG was about from 1. 0 to 3. 5 cm. Microscopically,10 cases showed typical features of SCSG with microcystic, follicular, papillary and solid structures with abundant extracellular mucinous secretions. The tumor cells had low-grade nuclei. Immunohistochemistry showed that all 10 tumors had diffuse and strong reactivities to cytokeratin 7,S-100 protein, mammaglobin, and 8 cases showed focal reactivity to cytokeratin 5/6,all were negative for Dog-1,CD117,p63 and calponin. Ki-67 was less than 5 perccent. FISH was performed in 10 cases of SCSG,including 8 cases with characteristic ETV6-NTRKS fusion gene and 2 cases with weak fluorescence signal. CONCLUSIONS Most salivary gland secretory carcinoma is a low-grade malignant tumor, which can be diagnosed according to its characteristic histological morphology and immunohistochemistry. The vast majority of cases were with ETV6-NTRK3 gene fusion, but its specific molecular classification remains to be further study.