McCluggage W Glenn, Aydin N Engin, Wong Newton A C S, Cooper Kumarasen
Department of Pathology, Royal Group of Hospitals Trust, Belfast, UK.
Int J Gynecol Pathol. 2009 May;28(3):286-91. doi: 10.1097/PGP.0b013e31818e1040.
We report 2 cases of a distinctive neoplasm arising from Bartholin gland and presenting as a vulval or vaginal mass. The tumors occurred in patients aged 44 and 51 years and were 2 and 3 cm in maximum dimension. In both cases, normal Bartholin gland tissue was identified adjacent to the lesion. The neoplasms were unencapsulated and largely well circumscribed but with a focally infiltrative edge. They were composed of tubular, trabecular, or insular arrangements with a double layer of inner cuboidal cells with round nuclei and outer cells with ovoid nuclei and clear cytoplasm, corresponding to epithelial and myoepithelial cells, respectively. Luminal eosinophilic colloid-like material was present. In both cases, a minor proportion of the neoplasm consisted of cribriform arrangements, creating an appearance reminiscent of adenoid cystic carcinoma, although the overall morphology was not typical of that lesion. Mitotic figures were identified in both cases, the mitotic count being 1 and 5/10 high-power fields. Immunohistochemically, the inner cells were positive with epithelial markers, including broad-spectrum cytokeratins and epithelial membrane antigen, and the outer cell layer was positive with myoepithelial markers p63, calponin, and alpha-smooth muscle actin. Both neoplasms exhibited diffuse strong immunoreactivity of the epithelial cells with c-kit. Activating mutations in KIT exons 9, 11, 13, and 17 and in platelet-derived growth factor receptor alpha exons 12, 14, and 18 were searched for by polymerase chain reaction and direct sequencing but were not identified. We believe this represents a low-grade carcinoma arising from Bartholin gland composed of a dual population of epithelial and myoepithelial cells and closely resembling the salivary gland neoplasm termed epithelial-myoepithelial carcinoma. We propose the term low-grade epithelial-myoepithelial carcinoma of Bartholin gland.
我们报告2例起源于巴氏腺并表现为外阴或阴道肿物的独特肿瘤。肿瘤发生于44岁和51岁的患者，最大直径分别为2 cm和3 cm。在这两例中，均在病变旁发现正常巴氏腺组织。肿瘤无包膜，大部分边界清晰，但边缘有局灶性浸润。它们由管状、小梁状或岛状结构组成，有双层细胞，内层为立方体细胞，核圆形，外层细胞的核呈卵圆形，胞质透明，分别对应上皮细胞和肌上皮细胞。管腔内有嗜酸性胶体样物质。在这两例中，肿瘤的一小部分由筛状结构组成，形成一种类似腺样囊性癌的外观，尽管总体形态并非该病变的典型表现。两例均可见核分裂象，核分裂计数分别为1/10高倍视野和5/10高倍视野。免疫组化显示，内层细胞对包括广谱细胞角蛋白和上皮膜抗原在内的上皮标记物呈阳性，外层细胞层对肌上皮标记物p63、钙调蛋白和α平滑肌肌动蛋白呈阳性。两例肿瘤的上皮细胞均对c-kit呈弥漫性强免疫反应。通过聚合酶链反应和直接测序检测KIT外显子9、11、13和17以及血小板衍生生长因子受体α外显子12、14和18中的激活突变，但未发现。我们认为这是一种起源于巴氏腺的低级别癌，由上皮细胞和肌上皮细胞双群体组成，与称为上皮-肌上皮癌的涎腺肿瘤极为相似。我们提出巴氏腺低级别上皮-肌上皮癌这一术语。
