Takeda Maiko, Tani Yoko, Saijo Nobuhiko, Shimizu Shigeki, Taniguchi Yoshihiko, Otsuka Kenji, Nakao Keiko, Tamiya Akihiro, Okishio Kyoichi, Atagi Shinji, Ohbayashi Chiho, Kasai Takahiko
National Hospital Organization Kinki-chuo Chest Medical Center, Sakai, Japan.
Osaka City University, Osaka, Japan.
Int J Surg Pathol. 2020 Feb;28(1):109-114. doi: 10.1177/1066896919870866. Epub 2019 Aug 25.
-deficient thoracic sarcoma (SMARCA4-DTS) is a recently described entity of thoracic sarcomas with an undifferentiated rhabdoid morphology and inactivation. Regardless of some reports about the histopathological findings so far, there have been only a few reports about the cytological features. In this article, we present the pathological features of 2 SMARCA4-DTS cases, including the cytological findings. Histopathologically, the tumor cells showed atypical loosely cohesive large epithelioid cells focally with geographic necrosis. Some cells were characterized by rhabdoid cells. Both patients showed intrathoracic masses with a history of smoking, and loss of SMARCA4 expression was confirmed with histopathological specimens. Immunohistochemically, tumor cells of both cases were at least focally positive for cytokeratin, CD34, CD99, synaptophysin, SOX2, and SALL4. In addition, tumor cells demonstrated significantly reduced expression of BRG1/SMARCA4 and SMARCA2. In conclusion, SMARCA4-DTS should be taken into consideration in the differential diagnosis of tumors with undifferentiated rhabdoid morphology involving the thoracic region.
SMARCA4缺陷型胸段肉瘤（SMARCA4-DTS）是一种最近描述的胸段肉瘤实体，具有未分化的横纹肌样形态和SMARCA4失活。尽管到目前为止有一些关于组织病理学发现的报道，但关于细胞学特征的报道却很少。在本文中，我们展示了2例SMARCA4-DTS病例的病理特征，包括细胞学发现。组织病理学上，肿瘤细胞表现为非典型的、松散聚集的大上皮样细胞，局灶性伴有地图状坏死。一些细胞具有横纹肌样细胞的特征。两名患者均表现为胸内肿块且有吸烟史，组织病理学标本证实存在SMARCA4表达缺失。免疫组化显示，两例病例的肿瘤细胞至少局灶性地对细胞角蛋白、CD34、CD99、突触素、SOX2和SALL4呈阳性。此外，肿瘤细胞显示BRG1/SMARCA4和SMARCA2的表达显著降低。总之，在鉴别诊断累及胸段区域的具有未分化横纹肌样形态的肿瘤时，应考虑SMARCA4-DTS。
