Essary Lydia R, Vargas Sara O, Fletcher Christopher D M
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Cancer. 2002 Jan 15;94(2):459-69. doi: 10.1002/cncr.10188.
Primary pleuropulmonary synovial sarcoma (SS) is a rare neoplasm and a recently recognized anatomic subset. Its clinicopathologic attributes are not yet well defined.
In this study, the clinical and histopathologic features of 12 SS arising in the lung and/or pleura were analyzed.
The neoplasms occurred in 7 men and 5 women, 20-72 years old (median, 31 years), were well circumscribed with a mean size of 7.2 cm, and involved either lung (9 cases), pleura (2 cases), or both (1 case). All the tumors were of monophasic type. Nine showed a classic spindle cell pattern, and three showed predominantly poorly differentiated features. All but one case showed at least focal positivity for epithelial membrane antigen (EMA), a finding characteristic of this tumor. The lack of EMA staining in one case, proven by electron microscopy to be SS, was attributed to the scarcity of material available for immunohistochemical stains. The diagnosis was proven cytogenetically in three cases. Within 2 years, local recurrence developed in 8 patients (75%), 3 of whom developed metastasis (25%). Five patients died of their disease within 2.5 years, 4 of them from uncontrolled local disease.
The authors conclude that pleuropulmonary SS, although rare, represents a distinct anatomic subset having pathologic features similar to those of its soft tissue counterpart. Its clinical behavior appears more aggressive, perhaps because of relatively later presentation combined with the difficulty in obtaining a wide surgical margin.
原发性胸膜肺滑膜肉瘤（SS）是一种罕见的肿瘤，也是最近才被认识的一个解剖学亚组。其临床病理特征尚未明确界定。
在本研究中，分析了12例发生于肺和/或胸膜的滑膜肉瘤的临床和组织病理学特征。
肿瘤发生于7名男性和5名女性，年龄在20至72岁之间（中位年龄31岁），边界清晰，平均大小为7.2 cm，累及肺（9例）、胸膜（2例）或两者（1例）。所有肿瘤均为单相型。9例表现为典型的梭形细胞模式，3例主要表现为低分化特征。除1例病例外，所有病例至少局灶性表达上皮膜抗原（EMA），这是该肿瘤的一个特征性表现。1例EMA染色阴性的病例经电镜证实为滑膜肉瘤，其原因是可用于免疫组化染色的材料稀缺。3例病例经细胞遗传学确诊。2年内，8例患者（75%）出现局部复发，其中3例发生转移（25%）。5例患者在2.5年内死于该病，其中4例死于无法控制的局部疾病。
作者得出结论，胸膜肺滑膜肉瘤虽然罕见，但代表了一个独特的解剖学亚组，其病理特征与其软组织对应物相似。其临床行为似乎更具侵袭性，可能是因为就诊相对较晚，加上难以获得较宽的手术切缘。
