Skálová Alena, Gnepp Douglas R, Lewis James S, Hunt Jennifer L, Bishop Justin A, Hellquist Henrik, Rinaldo Alessandra, Vander Poorten Vincent, Ferlito Alfio
*Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic †Department of Pathology, Providence, RI and Fall River, MA Departments of ‡Pathology, Microbiology, and Immunology §Otolaryngology, Vanderbilt University Medical Center, Nashville, TN ∥Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR ¶Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD #Department of Biomedical Sciences and Medicine, University of Algarve, Faro, Portugal **University of Udine School of Medicine, Udine, Italy ††Department of Otorhinolaryngology-Head and Neck Surgery, University Hospitals Leuven ‡‡Department of Oncology, Section Head and Neck Oncology, KU Leuven, Leuven, Belgium §§International Head and Neck Scientific Group, Padua, Italy.
Am J Surg Pathol. 2017 Aug;41(8):e33-e47. doi: 10.1097/PAS.0000000000000883.
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.
唾液腺可能引发多种不同的肿瘤。本综述聚焦于近期文献中已被认可为新肿瘤实体的4种唾液腺肿瘤：乳腺样分泌性癌、小唾液腺筛状腺癌（CASG）、硬化性多囊性腺病/腺瘤（SPA）以及肌上皮瘤的黏液性/分泌性变体。乳腺样分泌性癌是一种独特的低级别恶性唾液腺癌，具有特征性的染色体易位t(12;15)(p13;q25)，导致ETV6-NTRK3融合。筛状腺癌（CASG）是一种独特的肿瘤实体，与多形性低级别腺癌在位置（即最常发生于舌部）、显著的核空泡化、PRKD基因家族的不同改变以及原发性肿瘤出现时频繁转移的临床行为等方面存在差异。在大多数CASG病例中可见早期淋巴结转移疾病；然而，它们仍与惰性临床行为相关，使其成为所有低级别唾液腺肿瘤中的独特肿瘤。SPA是一种罕见的唾液腺硬化性肿瘤，其特征为囊性导管结构与包括空泡状、顶泌、黏液性、鳞状和泡沫状细胞在内的可变细胞内衬相结合，有突出的大腺泡细胞，其胞质有粗大嗜酸性的酶原样颗粒，以及紧密排列的导管结构，周围有外周肌上皮层和伴有局灶性炎症浸润的间质纤维化。SPA常伴有导管内上皮发育异常增殖，范围从轻度发育异常到重度发育异常/原位癌。此外，通过HUMARA分析已证明SPA是一个克隆过程，且与相当高的复发风险相关。因此，基于所有这些新认识到的发现，我们认为SPA可能是一种肿瘤，并建议命名为“硬化性多囊性腺瘤”。肌上皮瘤的黏液性变体是一种肌上皮肿瘤，有突出的胞质空泡化灶，常含有细胞内黏液物质并具有印戒形态。
