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目的探讨骨外黏液样软骨肉瘤的临床病理特征及鉴别诊断。方法应用HE、免疫组化染色观察3例骨外黏液样软骨肉瘤的组织学形态及免疫组化特征,并复习文献。结果3例中女性2例,男性1例,年龄30～52岁;2例发生于大腿,1例发生于手部,肿块均位于深部软组织。光镜下肿瘤呈多结节状,结节由纤维、疏松纤维血管组织分隔形成大小不等的小叶,结节内为丰富的黏液样基质,小叶周边区细胞丰富,中央区细胞稀疏;瘤细胞为圆形、卵圆形及短梭形,排列呈索状或吻合条索状;病灶内可见灶性出血,无坏死,未见明确软骨分化。免疫组化:肿瘤细胞vimentin均(+),2例NSE和Syn(+),1例S-100和CgA(+),1例EMA灶性(+);CK、HHF35、desmin、actin和GFAP均(-)。结论骨外黏液样软骨肉瘤是一种罕见的恶性软组织肿瘤,具有特征性的组织病理学特点,可伴有神经内分泌分化;虽然生长缓慢,但易转移和局部复发,需长期随访。
Objective To investigate the clinicopathological features and differential diagnosis of extraosseous mucoid chondrosarcoma. Methods HE and immunohistochemistry were used to observe the histological and immunohistochemical features of 3 cases of extranodal mucoid chondrosarcoma and to review the literature. Results In 3 cases, there were 2 females and 1 males, aged from 30 to 52 years old. Two cases occurred in the thigh and one case occurred in the hands. The tumors were located in deep soft tissues. Under the light microscope, the tumor was nodular. The nodules were separated by fibers and loose fiber blood vessels to form the leaflets of different sizes. The nodules were rich in myxoid matrix. The cells in the peripheral lobules were rich in cells and the cells in the central area were sparse. Round, oval and short fusiform, arranged in a cord-like or anastomotic labyrinth; focal lesions visible focal hemorrhage, no necrosis, no clear cartilage differentiation. Immunohistochemistry: Vimentin (+), two NSE and Syn (+), one S-100 and CgA (+) and one EMA focal (+); CK, HHF35, desmin, actin and GFAP all(-). Conclusions Extraosseous mucoid chondrosarcoma is a rare malignant soft tissue tumor with characteristic histopathological features, which may be associated with neuroendocrine differentiation. Although it grows slowly, metastasis and local recurrence require long-term follow-up.