Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston, TX, USA.
Department of Otorhinolaryngology-Head and Neck Surgery, University of Texas Health Science Center at Houston, TX, USA.
Int J Surg Pathol. 2020 Oct;28(7):782-786. doi: 10.1177/1066896920913109. Epub 2020 Apr 3.
Desmoplastic small round cell tumor (DSRCT) is a rare malignant mesenchymal neoplasm that typically affects young patients and presents as large intraabdominal masses. We report a rare case of parotid gland DSRCT in a 38-year-old man. The tumor cells were large sized and form irregular nests embedded in abundant desmoplastic stroma. Immunohistochemically, the tumor cells were positive for cytokeratin, GATA3, p40, and p63. Working differential diagnosis included squamous cell carcinoma, myoepithelial carcinoma, hyalinizing clear cell carcinoma, NUT (nuclear protein of the testis) carcinoma, and adamantinoma-like Ewing sarcoma. The characteristic morphologic features and presence of gene fusion are diagnostic of DSRCT. Salivary gland DSRCT is an exceedingly rare entity, with only 6 cases reported in the literature.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的恶性间叶性肿瘤，主要发生于年轻患者，表现为巨大的腹腔内肿块。我们报告了 1 例罕见的腮腺 DSRCT 病例，患者为 38 岁男性。肿瘤细胞体积较大，呈不规则巢状排列，嵌入丰富的促结缔组织增生性间质中。免疫组织化学染色显示，肿瘤细胞表达细胞角蛋白、GATA3、p40 和 p63。鉴别诊断包括鳞状细胞癌、肌上皮癌、透明细胞癌、NUT（睾丸核蛋白）癌和类似于造釉细胞瘤的尤文肉瘤。特征性的形态学特征和基因融合的存在是诊断 DSRCT 的依据。涎腺 DSRCT 极为罕见，文献中仅报道了 6 例。
