Zámecník M, Michal M, Gomolcák P
Katedra patológie SPAM, Bratislava.
Cesk Patol. 1999 Jul;35(3):90-3.
We describe a case of extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation. The tumor occurred in subcutaneous tissue of the right popliteal region in a 50-year-old man. It measured 5 cm in diameter, was well circumscribed, lobular and gelatinous, and lacked any necrosis or hemorrhage. Histologically, the tumor structure was a typical of extraskeletal myxoid chondrosarcoma. The lesion was lobulated and contained small to medium-sized chondroblast-like cells with ovoid hyperchromatic nuclei and without prominent nucleoli. The cells created cords and nests and showed focally a perivascular rosette-like arrangement. A few of the tumor cells were spindle shaped. The myxoid matrix was stained with alcian blue and this reaction was resistant to prior treatment with hyaluronidase. PAS-positive glycogen was found in the cytoplasm of some tumor cells. Immunohistochemically, the tumor cells were diffusely positive for neuron specific enolase, monoclonal synaptophysin and vimentin. Following antibodies gave negative results: desmin, actins, S-100 protein, pancytokeratin, epithelial membrane antigen, chromogranin A, neurofilament protein, myelinic basic protein, glial fibrillary acidic protein. The patient is well four years after the wide excision of tumor and radiotherapy. Neuroendocrine differentiation in extraskeletal myxoid chondrosarcoma was described at first by Chhieng et al. in 1998 (1). Our observation confirms this interesting finding.
我们报告一例具有神经内分泌分化的骨外黏液样软骨肉瘤。该肿瘤发生于一名50岁男性右腘窝区域的皮下组织。肿瘤直径5厘米，边界清晰，呈分叶状且质地胶冻样，无坏死或出血。组织学上，肿瘤结构为典型的骨外黏液样软骨肉瘤。病变呈分叶状，包含小至中等大小的成软骨细胞样细胞，核呈卵圆形、深染，无明显核仁。细胞形成条索状和巢状结构，局部呈血管周围玫瑰花结样排列。少数肿瘤细胞呈梭形。黏液样基质经阿尔辛蓝染色，该反应对预先用透明质酸酶处理具有抗性。在一些肿瘤细胞的细胞质中发现PAS阳性糖原。免疫组化显示，肿瘤细胞对神经元特异性烯醇化酶、单克隆突触素和波形蛋白呈弥漫性阳性。以下抗体检测结果为阴性：结蛋白、肌动蛋白、S-100蛋白、全细胞角蛋白、上皮膜抗原、嗜铬粒蛋白A、神经丝蛋白、髓鞘碱性蛋白、胶质纤维酸性蛋白。该患者在肿瘤广泛切除及放疗后四年情况良好。骨外黏液样软骨肉瘤的神经内分泌分化最初由Chhieng等人于1998年描述(1)。我们的观察证实了这一有趣的发现。
