富含细胞外黏液的唾液腺肿瘤诊断及鉴别诊断OA
Diagnosis and differential diagnosis of mucin-rich salivary gland tumors
本文系统综述了以大量细胞外黏液为主要或显著特征的唾液腺肿瘤诊断及鉴别诊断要点,明确了核心鉴别特征."富含细胞外黏液"在此特指黏液成为肿瘤的主要构成成分,而非局灶性或少量存在,这种现象与独特的组织发生学机制相关:一方面源于特定基因突变(如黏液腺癌中的AKT1 E17K)促使导管上皮分化为黏液细胞并大量分泌黏液;另一方面则源于肌上皮细胞分泌糖胺聚糖形成黏液样间质.含大量细胞外黏液的唾液腺肿瘤包括黏液性囊腺瘤、乳头状涎腺瘤样导管内乳头状瘤、黏液性肌上皮瘤、间质富含黏液的多形性腺瘤、黏液腺癌、低级别黏液表皮样癌、富黏液型唾液腺导管癌及肠型腺癌.此类肿瘤在诊断上面临双重挑战:大量黏液既可作为某些肿瘤的典型特征,也可能在其他肿瘤中掩盖其诊断性结构,导致组织学形态重叠与特征区域隐匿.核心鉴别要点包括:组织学上需仔细辨识被黏液掩盖的典型结构(如黏液表皮样癌中的表皮样细胞、唾液腺导管癌的大汗腺特征);在免疫组化方面,应用CK20可鉴别肠型腺癌(阳性)与黏液腺癌(阴性),而应用雄激素受体可以鉴别唾液腺导管癌(阳性)与黏液表皮样癌(阴性);分子检测对确诊具有关键作用(如AKT1 E17K突变见于黏液腺癌,MAML2重排见于黏液表皮样癌,MEF2C::SS18融合见于微分泌性腺癌).本文系统梳理了富含细胞外黏液唾液腺肿瘤的核心病理特征与鉴别要点,以期为临床病理诊断提供实用参考.
This paper systematically elaborates on the key points of diagnosis and differential diagnosis of salivary gland tumors characterized by a substantial amount of extracellular mucus as a main or prominent feature,and clarifies the core differential features.The term"mucus-rich"specifically denotes that mucus is a major component of the tumor,rather than a focal or minor one.This phenomenon is associated with distinct histogenetic mechanisms:it may result from specific genetic mutations(e.g.,AKT1 E17K in mucinous adenocarcinoma)that drive ductal epithelial differentia-tion into mucus-secreting cells,or from myoepithelial cells secreting glycosaminoglycans that form a myxoid stroma.Salivary gland tumors with abundant extracellular mucus include mucinous cystadenoma,sialadenoma papilliferum-like intraductal papillary tumors,mucinous myoepithelioma,pleomorphic adenoma with mucin-rich stroma,mucinous adeno-carcinoma,low-grade mucoepidermoid carcinoma,mucin-rich salivary duct carcinoma and intestinal-type adenocarci-noma.The diagnosis of these tumors is complicated by the dual nature of extracellular mucus:while it is a defining fea-ture of some entities,it can also obscure key diagnostic architectural features in others,leading to histological overlap and inconspicuous diagnostic areas.Given the frequent histological morphological overlap among these tumors,immuno-histochemical findings and molecular characteristics have emerged as crucial differential diagnostic criteria.Core differ-ential diagnostic points include the following:histologically,there must be meticulous identification of typical structures obscured by mucin(such as squamoid cells in mucoepidermoid carcinoma and apocrine features in salivary duct carci-noma);in immunohistochemical staining,CK20 is useful for distinguishing intestinal-type adenocarcinoma(positive)from mucinous adenocarcinoma(negative),while androgen receptor aids in differentiating salivary duct carcinoma(posi-tive)from mucoepidermoid carcinoma(negative);and molecular testing plays a critical role in definitive diagnosis(e.g.,the AKT1 E17K mutation for mucinous adenocarcinoma,MAML2 rearrangement for mucoepidermoid carcinoma,and MEF2C::SS18 fusion for microsecretory adenocarcinoma).This paper systematically summarizes the core pathological features and differential diagnostic points of mucin-rich salivary gland tumors,aiming to provide a practical reference for clinical pathological diagnosis.
关苇杭;刘苍维;郭昊;李金薇;王丹丹;乔春燕;聂孟冬;曲明;史册
吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)吉林大学口腔医院口腔病理科,吉林省牙发育及颌骨重塑与再生重点实验室,吉林 长春(130021)
医药卫生
唾液腺肿瘤黏液诊断黏液腺癌黏液性囊腺瘤多形性腺瘤黏液表皮样癌肌上皮瘤免疫组织化学病理学
salivary gland tumorsmucusdiagnosismucinous adenocarcinomamucinous cystadenomapleomorphic adenomamucoepidermoid carcinomamyoepitheliomaimmunohistochemistrypathology
《口腔疾病防治》 2026 (6)
606-619,14
吉林省财政厅科技项目(JCSZ2021893-36)吉林省自然科学基金项目(YDZJ202201ZYTS039)中央高校基本科研业务费专项资金 This study was supported by the grants from Jilin Province Department of Finance(No.JCSZ2021893-36),Jilin Prov-ince Natural Science Foundation Project(No.YDZJ202201ZYTS039)and Special Funds for Basic Research of Central Universities.
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