Payen L, Pluot M, Delhomme H, Mourad H, Kalis B, Caulet T
Ann Pathol. 1983 Sep;3(3):245-9.
The authors report the case of a labial embryonal rhabdomyosarcoma of a baby. This appears to be an exceptional localization. The study of the first biopsy which was too superficial leads to the thought of a capillary angioma. At the age of 6 months the lesion is 35 mm in diameter, a lobulated formation, typically botryoid. The excision after a frozen section control was in healthy tissue. At the age of 10 months, there is a local relapse with a nodule of 18 mm with left submandibular lymph nodes. A second surgical operation allows the culture of the tumoral tissue. The conventional histopathological examinations shows the aspect of an embryonal rhabdomyosarcoma. On the ultrastructural examination one can visualize in some cells an intracytoplasmic filamentous material. The cellular proliferation in culture, after May Grunwald Giemsa coloration is monomorphous, spindle shaped and of fibroblastic aspect. The ultrastructural study of this cellular proliferation after trypsinization on the 7th day shows some cells including an intracytoplasmic filamentous material. On the data of the cellular culture, the ultrastructural studies and the review of the literature, the authors discuss the possibility of the relationship between the cells of embryonal rhabdomyosarcomas and the fibroblasts and myofibroblasts. Embryonal sarcoma seems to be a better denomination than embryonal rhabdomyosarcoma, as regards the histogenesis.
作者报告了一例婴儿唇部胚胎性横纹肌肉瘤的病例。这似乎是一种罕见的发病部位。首次活检过于表浅，结果让人误以为是毛细血管瘤。6个月大时，病灶直径为35毫米，呈分叶状，典型的葡萄状。在冰冻切片检查后进行切除，切除范围在健康组织内。10个月大时，出现局部复发，有一个18毫米的结节，并伴有左下颌下淋巴结肿大。第二次手术获取了肿瘤组织用于培养。传统组织病理学检查显示为胚胎性横纹肌肉瘤的表现。超微结构检查发现一些细胞内可见胞质丝状物质。培养的细胞经May Grunwald Giemsa染色后，细胞增殖呈单形性，纺锤形，具有成纤维细胞样外观。第7天胰蛋白酶消化后对这种细胞增殖进行超微结构研究，发现一些细胞内含有胞质丝状物质。基于细胞培养数据、超微结构研究以及文献回顾，作者探讨了胚胎性横纹肌肉瘤细胞与成纤维细胞及肌成纤维细胞之间存在关联的可能性。就组织发生而言，胚胎性肉瘤似乎比胚胎性横纹肌肉瘤是一个更好的命名。
