Ceballos Sáenz Celina, Argyris Prokopios P, Manivel J Carlos, Urias Barreras Cynthia Marina, Koutlas Ioannis G
National Autonomous University of Mexico, Mexico City, Mexico.
University of Minnesota, Minneapolis, MN, USA.
Int J Surg Pathol. 2014 Oct;22(7):667-72. doi: 10.1177/1066896914526778. Epub 2014 Mar 11.
Hyalinizing clear cell carcinoma (HCCC) is a rare low-grade malignant tumor affecting the minor salivary glands; nasopharyngeal involvement is uncommon.
A 38-year-old male patient presented with a 3.2 × 4.5 × 4.4 cm expansile mass obliterating the lumen of the nasopharynx and extending into the left nasal cavity. Histopathologically, the tumor was characterized by clear round to polygonal epithelial cells arranged in anastomosing trabeculae and solid nests. The stroma consisted of fibromyxoid connective tissue with areas of intense hyalinization and desmoplasia. Immunohistochemically, strong and diffuse reactivity for AE1/AE3, CK5/6, and p63 was observed. EWSR1 gene rearrangement was confirmed by fluorescence in situ hybridization. The diagnosis of nasopharyngeal HCCC was rendered. Surgical excision was performed along with adjuvant radiotherapy and chemotherapy.
HCCC generally demonstrates good prognosis with low metastatic potential. Identification of EWSR1 gene disruption is usefulin discerning HCCC from other neoplasms with overlapping microscopic features.
透明细胞癌（HCCC）是一种罕见的低度恶性肿瘤，累及小唾液腺；鼻咽部受累并不常见。
一名38岁男性患者，出现一个3.2×4.5×4.4 cm的膨胀性肿块，阻塞鼻咽腔并延伸至左侧鼻腔。组织病理学上，肿瘤的特征是透明的圆形至多边形上皮细胞，排列成交错的小梁和实性巢状结构。间质由纤维黏液样结缔组织组成，有明显的玻璃样变和促纤维组织增生区域。免疫组化显示，AE1/AE3、CK5/6和p63呈强弥漫性反应。荧光原位杂交证实了EWSR1基因重排。诊断为鼻咽部HCCC。进行了手术切除，并辅助放疗和化疗。
HCCC一般预后良好，转移潜能低。EWSR1基因破坏的鉴定有助于将HCCC与其他具有重叠微观特征的肿瘤区分开来。
