Primary pleuropulmonary synovial sarcoma - Reappraisal of a recently described anatomic subset
Primary pleuropulmonary synovial sarcoma - Reappraisal of a recently described anatomic subset
复制标题
DOI：
10.1002/cncr.10188
复制
发表时间：
2002-01-15
期刊：
影响因子：
6.2
通讯作者：
Fletcher, CDM
中科院分区：
文献类型：
--
作者：
Essary, LR;Vargas, SO;Fletcher, CDM
背景资料。原发性胸膜肺滑膜肉瘤(SS)是一种罕见的肿瘤，是新近认识到的一个解剖亚型。方法：分析12例肺和/或胸膜SS的临床和病理特征。结果：肿瘤发生于男性7例，女性5例，年龄20~72岁(中位数31岁)，平均大小7.2 cm，累及肺部9例，胸膜2例，或两者兼有1例。肿瘤均为单相型。其中9例表现为典型的梭形细胞形态，3例表现出明显的低分化特征。除1例外，所有病例均显示上皮膜抗原(EMA)局灶性阳性，这是该肿瘤的特征。1例经电子显微镜证实为SS的病例缺乏EMA染色，其原因是免疫组织化学染色材料稀少。3例经细胞遗传学证实，2年内局部复发8例(75%)，其中3例发生转移(25%)。5例患者在2.5年内死于本病，其中4例死于未控制的局部疾病。结论作者认为，胸膜肺SS虽然罕见，但具有与软组织相似的病理特征。它的临床表现似乎更具侵袭性，可能是因为相对较晚的表现加上很难获得广泛的手术切缘。(C)2002年美国癌症协会。
BACKGROUND. Primary pleuropulmonary synovial sarcoma (SS) is a rare neoplasm and a recently recognized anatomic subset. Its clinicopathologic attributes are not yet well defined.METHODS. In this study, the clinical and histopathologic features of 12 SS arising in the lung and/or pleura were analyzed.RESULTS. 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.CONCLUSIONS. 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. (C) 2002 American Cancer Society.