Weidner N, Goldman R, Johnston J
Department of Pathology, University of California at San Francisco 94143.
Ultrastruct Pathol. 1993 May-Aug;17(3-4):287-94. doi: 10.3109/01913129309027775.
A 47-year-old man presented with a soft tissue mass of the distal right thigh near the knee. The tumor was highly vascular with epithelioid tumor cells growing in a peritheliomatous pattern, suggesting a soft tissue glomus tumor. Yet many tumor cells contained hemosiderin pigment and formed papillary structures suggestive of pigmented villonodular synovitis. Tumor cells were cytologically bland, and there was minimal mitotic activity. The tumor cells were strongly immunoreactive for cytokeratin, however, and contained true desmosomes, gland lumina, microvilli, tonofilaments, and well-developed basal lamina. These findings plus the absence perinuclear aggregates of intermediate filaments rule out malignant rhabdoid tumor and epithelioid sarcoma. Also, magnetic resonance imaging revealed no other lesions to suggest metastatic carcinoma. Thus this tumor appears to be a predominantly epithelioid form of monophasic synovial sarcoma. Recognition of this variant of synovial sarcoma is important for prognostication and therapeutic decision making because some studies indicate that this variant of synovial sarcoma follows a relatively benign clinical course.
一名47岁男性患者，右大腿远端靠近膝关节处出现软组织肿块。肿瘤血管丰富，上皮样肿瘤细胞呈血管球瘤样生长，提示为软组织血管球瘤。然而，许多肿瘤细胞含有含铁血黄素色素，并形成乳头状结构，提示色素沉着绒毛结节性滑膜炎。肿瘤细胞在细胞学上表现温和，有丝分裂活动极少。肿瘤细胞对细胞角蛋白呈强免疫反应，并且含有真正的桥粒、腺腔、微绒毛、张力丝和发育良好的基底膜。这些发现加上中间丝无核周聚集排除了恶性横纹肌样瘤和上皮样肉瘤。此外，磁共振成像未发现其他提示转移性癌的病变。因此，该肿瘤似乎是单相滑膜肉瘤的主要上皮样形式。认识这种滑膜肉瘤变体对于预后评估和治疗决策很重要，因为一些研究表明这种滑膜肉瘤变体的临床病程相对良性。
