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Newly Described Entities in Salivary Gland Pathology.

新发现的涎腺肿瘤类型

Skálová A,Gnepp DR,Lewis JS,Hunt JL,Bishop JA,Hellquist H,Rinaldo A,Vander Poorten V,Ferlito A

Abstract

Salivary glands may give rise to a wide spectrum of different tumors. This review concentrates on 4 salivary gland tumors that have been accepted in the recent literature as new neoplastic entities: mammary analog secretory carcinoma, cribriform adenocarcinoma of minor salivary glands (CASG), sclerosing polycystic adenosis/adenoma (SPA), and the mucinous/secretory variant of myoepithelioma. Mammary analog secretory carcinoma is a distinctive low-grade malignant salivary cancer that harbors a characteristic chromosomal translocation, t(12;15) (p13;q25), resulting in an ETV6-NTRK3 fusion. Cribriform adenocarcinoma (CASG) is a distinct tumor entity that differs from polymorphous low-grade adenocarcinoma by location (ie, most often arising on the tongue), by prominent nuclear clearing, differing alterations of the PRKD gene family, and clinical behavior with frequent metastases at the time of presentation of the primary tumor. Early nodal metastatic disease is seen in most cases of CASG; yet, they are still associated with indolent clinical behavior, making it a unique neoplasm among all low-grade salivary gland tumors. SPA is a rare sclerosing tumor of the salivary glands characterized by the combination of cystic ductal structures with variable cell lining including vacuolated, apocrine, mucinous, squamous, and foamy cells, by prominent large acinar cells with coarse eosinophilic cytoplasmic zymogen-like granules, and by closely packed ductal structures, surrounded by a peripheral myoepithelial layer and stromal fibrosis with focal inflammatory infiltrates. SPA frequently harbors intraductal epithelial dysplastic proliferations ranging from mild dysplasia to severe dysplasia/carcinoma in situ. Moreover, SPA has been proven to be a clonal process by HUMARA assay and is associated with considerable risk of recurrence. Therefore, on the basis of all these newly recognized findings, we believe that SPA is likely a neoplasm, and we suggest the name "sclerosing polycystic adenoma." The mucinous variant of myoepithelioma is a myoepithelial tumor with foci of prominent cytoplasmic clearing frequently containing intracellular mucin material and having signet-ring morphology.

摘要

涎腺肿瘤谱多而广。本文重点综述4文献认为是类型涎腺瘤:包括类似乳腺分泌型、小涎腺筛状腺癌( CASG)、硬化型多囊性腺病/腺瘤、肌上皮瘤的粘液/分泌型。类似乳腺分泌型是一种特殊的低度恶性涎腺肿瘤,具有特征性的染色体t (12;15 ) (p13;q25 )异常导致的融合基因ETV6-NTRK3表达。筛状腺癌(CASG)是一种特殊肿瘤类型不同于特定部位发生的多形性低度恶性腺癌(最常发生在),具有不同于PRKD基因谱系肿瘤特征的透明细胞核,且临床上原发肿瘤发现时已发生转移。大多数CASG早期就存在淋巴结转移,尽管如此,它们仍然具有惰性的临床行为,这一特点使它成为低级别涎腺肿瘤中独特的类型SPA是一种罕见的具有硬化特点、囊性导管结构的涎腺肿瘤囊性导管结构管壁覆多种类型细胞,包括空泡顶浆分泌的、粘液、鳞状泡沫细胞,多数为胞浆内含嗜酸性酶原颗粒的大腺泡细胞,导管外周为紧密包绕的肌上皮细胞层伴灶状炎细胞浸润的硬化性间质。SPA中的导管上皮常伴有不典型增生,从轻度到重度不典型增生原位癌。此外,HUMARA测定法证实证实SPA是一克隆性病变,并具有相当大的复发风险。因此,基于以上这些最新的认识及发现,我们认为SPA可能是一种肿瘤,建议命名为硬化性多囊腺瘤”。肌上皮瘤的粘液亚型是一种瘤细胞胞浆透明,细胞内常含有粘液、呈印戒样形态的肌上皮肿瘤。


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