Niwa Hideki, Masuda Shinji, Kobayashi Chikashi, Oda Yoshinao
Department of Pathology, Kouseiren Takaoka Hospital, Takaoka, Toyama, Japan.
Pathol Int. 2004 Aug;54(8):611-5. doi: 10.1111/j.1440-1827.2004.01670.x.
A rare case of primary pulmonary synovial sarcoma with polypoid endobronchial growth in a 42-year-old Japanese woman is described. Left upper sleeve lobectomy was performed for the polypoid tumor measuring 2.5 cm in the left major bronchus and the patient was treated with adjuvant chemotherapy. Three years later, a recurrent tumor was discovered. Microscopically, this tumor was characterized by a proliferation of oval to spindle-shaped cells arranged in sheets and fascicles and covered by the thin normal bronchial epithelium. Immunohistochemically, tumor cells were positive for vimentin, and focally positive for pancytokeratin recognized by AE1/AE3, cytokeratin 7 and epithelial membrane antigen. A chimera gene, SYT-SSX1, was detected. Recently, primary pulmonary synovial sarcoma is an increasingly recognized clinical entity; however, most of these tumors present as a parenchymal mass. The present case is a unique example of primary synovial sarcoma of endobronchial polypoid type. This case suggests that pulmonary synovial sarcoma might originate from bronchial submucosal stromal tissue.
本文描述了一名42岁日本女性原发性肺滑膜肉瘤伴息肉样支气管内生长的罕见病例。对位于左主支气管内、大小为2.5 cm的息肉样肿瘤实施了左上袖状肺叶切除术，并对患者进行了辅助化疗。三年后，发现了复发性肿瘤。显微镜下，该肿瘤的特征是椭圆形至梭形细胞呈片状和束状增殖，并被薄的正常支气管上皮覆盖。免疫组化显示，肿瘤细胞波形蛋白阳性，被AE1/AE3识别的全细胞角蛋白、细胞角蛋白7和上皮膜抗原局灶性阳性。检测到一种嵌合基因SYT-SSX1。近年来，原发性肺滑膜肉瘤是一种越来越被认可的临床实体；然而，这些肿瘤大多表现为实质肿块。本病例是支气管内息肉样型原发性滑膜肉瘤的一个独特例子。该病例提示肺滑膜肉瘤可能起源于支气管黏膜下间质组织。
