Int J Stomatol ›› 2025, Vol. 52 ›› Issue (2): 257-262.doi: 10.7518/gjkq.2025021

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Clinical pathological study on the microsecretory adenocarcinoma of salivary glands

Weiping Jie(),Ji’an Hu,Yining Li()   

  1. Dept. of Pathology, Stomatology Hospital, School of Stomatology, Zhejiang University School of Medicine, Zhejiang Provincial Clinical Research Center for Oral Diseases, Key Laboratory of Oral Biomedical Research of Zhejiang Province, Cancer Center of Zhejiang University, Engineering Research Center of Oral Biomaterials and Devices of Zhejiang Pro-vince, Hangzhou 310000, China
  • Received:2024-05-20 Revised:2024-07-23 Online:2025-03-01 Published:2025-03-01
  • Contact: Yining Li E-mail:jieweiping123@163.com;liyn@zju.edu.cn
  • Supported by:
    Medical Health Science and Technology Program Project in Zhejiang Province(2022516638);Zhejiang Provincial Department of Education General Research Project(Y202147707)

Abstract:

Microsecretory adenocarcinoma (MSA) is a new entity in the 5th edition of the Word Health Organization (WHO) Classification of Head and Neck Tumors. It is a low-grade malignant epithelial tumor of the salivary gland and has unique histological characteristics, immunoprofile, and molecular features. Histologically, MSA shows many growth patterns, including microcystic, cribriform, tubular, and cords, with abundant basophilic secretions in the lumens. The tumor is composed of a single population of intercalated duct-like cells. The stroma is fibromyxoid. For its immunoprofile, MSA is positive for SOX10, S100, and p63 and negative for p40, calponin, and mammaglobin. Meanwhile, MSA is va-riable for smooth muscle actin and exhibits a specific MEF2C∶ ∶SS18 gene fusion. The differential diagnosis for MSA includes secretory carcinoma, sclerosing microcystic adenocarcinoma, polymorphic adenocarcinoma, adenoid cystic carcinoma, and secretory myoepithelial carcinoma.

Key words: microsecretory adenocarcinoma, salivary tumor, MEF2C∶ ∶SS18 gene fusion

CLC Number: 

  • R739.87

TrendMD: 

Tab 1

MSA reported in the literature"

作者例数性别年龄/岁部位最大直径治疗随访
Bishop等[2,11]24女性13例、男性11例17~8314例腭部、6例颊部、2例磨牙后区、1例下颌角、1例腮腺0.6~3.0 cm17例手术切除,其余不详14例有随访信息者均无复发或转移,其余不详
Jurmeister等[13]162颊部原发灶不详,复发灶2 cm,肺部转移灶最大者1.2 cm手术切除2009年首次手术,2016年局部复发,2021年肺部结节手术,确定为肺部转移
Gui等[14]170上颌骨5.3 cm手术切除+化学治疗随访无复发或转移
Kawakami等[12]137腭部1.5 cm手术切除不详
贺晓娟等[3]127颊部2.2 cm手术切除随访无复发或转移
安风仙等[4]148腭部1.2 cm手术切除不详
Bishop等[6]461~741例鼻部皮肤、1例颏部皮肤、1例头皮、1例外耳道不详颏部及外耳道者手术切除,其余不详2例有随访信息者均无复发或转移,其余不详
Bogiatzi等[7]3女性2例、男性1例53~781例前臂皮肤、1例无名指皮肤、1例前顶叶区0.9~1.3 cm手术切除随访无复发或转移
Dibbern等[9]189外耳道不详手术切除不详
Chan等[8]144外耳道1.9 cm手术切除随访无复发或转移

Tab 2

Differential diagnosis of MSA"

肿瘤类别组织学特点生物学行为免疫组织化学表型分子遗传特点
MSA肿瘤排列呈微囊型、筛状、小管样和条索样,囊腔或管腔内含丰富的嗜碱性分泌物;为单相性肿瘤,缺乏肌上皮细胞和基底细胞分化;肿瘤间质为纤维样或黏液样组织肿瘤无明显包膜,有局部侵袭性,神经侵犯罕见阳性:SOX10、S100、p63;阴性:p40、calponin、mammaglobin;可变:SMAMEF2C-SS18基因融合
分泌性癌肿瘤排列呈微囊状、实性巢状、小管状、滤泡状或乳头囊状结构,管腔内含嗜酸性分泌物;肿瘤呈单相性,无肿瘤性肌上皮细胞肿瘤呈分叶状,小叶间有纤维间隔,常浸润性生长,部分病例边界可清楚阳性:S100、SOX10、mammaglobin、pan-TRK;阴性:DOG1、P63、SMA、calponinETV6-NTRK3融合
硬化性微囊性腺癌肿瘤排列呈小管状、小条索状、巢状,管腔内含嗜酸性分泌物;肿瘤呈双相性,由肿瘤性导管上皮细胞和肌上皮细胞构成;肿瘤间质常硬化肿瘤呈浸润性生长,神经侵犯多见阳性:CK7、S100、p63、p40
多形性腺癌肿瘤排列呈小叶状、乳头状或乳头囊状、条索状、筛状和小管状结构,肿瘤周边常见“溪流样”改变;肿瘤局部还可见嗜酸细胞、透明细胞、鳞状细胞或黏液细胞;为双相性肿瘤,由肿瘤性导管上皮细胞和肌上皮细胞构成肿瘤呈浸润性生长,神经侵犯常见阳性:CK7、S100、p63、SMA、 calponin;阴性: p40PRKD1、PRKD2、PR-KD3重排;PRKD1激活突变
腺样囊性癌肿瘤排列呈筛状、管状、实性结构,管腔内含有嗜酸性或嗜碱性肌上皮细胞分泌物;为双相性肿瘤,由肿瘤性导管上皮细胞和肌上皮细胞构成;肿瘤间质常有玻璃样变肿瘤浸润性生长,神经侵犯常见阳性:CK7、p63、p40、Myosin、SMA、panTRK

MYB-NFIB或MYBL1-

NFIB基因融合

分泌性肌上皮癌肿瘤排列呈片状、小梁状、条索状;多数肿瘤细胞内出现标志性的单个圆形、偏位的胞浆内黏蛋白空泡;有些肿瘤细胞内出现含有嗜酸性分泌物的空泡;不含分泌物的肿瘤性浆细胞样及上皮样肌上皮细胞也常可见边界清,以推挤型浸润为主,偶见广泛性浸润并伴有神经侵犯的病例阳性:肌上皮标记物,如calponin、SMA、p40、p63、S100PTEN/PI3K/AKT通路改变
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