Gaertner E, Zeren E H, Fleming M V, Colby T V, Travis W D
Department of Pathology, Walter Reed Army Medical Center, Washington, D.C., USA.
Am J Surg Pathol. 1996 Jan;20(1):36-45. doi: 10.1097/00000478-199601000-00004.
Five cases of primary synovial sarcoma of the pleura are presented with a discussion of differentiation from other biphasic malignant neoplasms, most notably malignant mesothelioma. The cases have clinical, histologic, and immunohistochemical features consistent with synovial sarcoma of soft tissue. The average age at initial presentation of the reported patients was 25 years with an approximate range of 9 to 50 years. A large pleural-based intrathoracic mass was identified in each case. Histologic analysis showed a biphasic (mixed) pattern composed of epithelial and spindle cells. The epithelial cells showed cleft-like to tubulopapillary growth with focal intraluminal periodic acid Schiff's (PAS)-positive, diastase-resistant secretions identified in four of the five cases. The spindle cell component was composed predominantly of densely packed, elongated, fusiform cells. Immunohistochemical staining showed positivity with antibodies against cytokeratin, BER.EP4, epithelial membrane antigen, and vimentin in all cases. The patients seem to have an aggressive course, with four deaths reported within 3 years from initial surgery. These cases represent the first reported cases of primary synovial sarcoma of the pleura and lend further credence to the theory that synovial sarcomas are derived from immature mesenchymal elements, not from synovium.
本文报告了5例原发性胸膜滑膜肉瘤，并讨论了其与其他双相性恶性肿瘤（最显著的是恶性间皮瘤）的鉴别诊断。这些病例具有与软组织滑膜肉瘤一致的临床、组织学和免疫组化特征。报告患者的初次就诊平均年龄为25岁，大致范围为9至50岁。每例均发现一个以胸膜为基底的巨大胸腔内肿块。组织学分析显示为双相（混合）模式，由上皮细胞和梭形细胞组成。上皮细胞呈裂隙样至微管乳头状生长，5例中有4例在管腔内发现局灶性高碘酸希夫（PAS）阳性、抗淀粉酶分泌物。梭形细胞成分主要由密集排列的细长梭形细胞组成。免疫组化染色显示，所有病例中细胞角蛋白、BER.EP4、上皮膜抗原和波形蛋白抗体均呈阳性。患者病程似乎较为凶险，据报告4例在初次手术后3年内死亡。这些病例是首次报告的原发性胸膜滑膜肉瘤病例，进一步支持了滑膜肉瘤起源于未成熟间充质成分而非滑膜的理论。
