Guedes Alex, Barreto Bruno G, Barreto Lara G S, de Oliveira Araújo Iguaracyra B, Queiroz Aristides C, Athanazio Daniel A, Athanazio Paulo R F
Department of Musculoskeletal Cancer Surgery, Santa Izabel Hospital, Salvador, Brazil.
J Cutan Pathol. 2009 Feb;36(2):270-3. doi: 10.1111/j.1600-0560.2008.01032.x.
Tumors traditionally classified as parachordomas are rare, usually indolent, soft tissue neoplasms. This case report describes a 6-year-old girl with a mass in her left forearm measuring 11 x 6 cm. Foci of moderate nuclear atypia and various areas of necrosis were found in the tumor. Lung metastases were detected 3 months after diagnosis. Immunophenotyping revealed a strong, diffuse expression of pancytokeratin, S-100 protein, vimentin, glial fibrillary acidic protein and focal expression of chromogranin A. This is the fifth documented report on cases of metastasized parachordomas. It is rare for these neoplasms to show such aggressive behavior; moreover, immunoreactivity to chromogranin A is unexpected in these tumors. The relationship between the so-called parachordomas and soft tissue myoepithelioma/mixed tumors is discussed, including the recently defined pediatric myoepithelial carcinoma.
传统上归类为副脊索瘤的肿瘤是罕见的、通常生长缓慢的软组织肿瘤。本病例报告描述了一名6岁女童，其左前臂有一个大小为11×6厘米的肿块。肿瘤中发现了中度核异型性灶和多个坏死区域。诊断后3个月检测到肺转移。免疫表型分析显示全细胞角蛋白、S-100蛋白、波形蛋白、胶质纤维酸性蛋白呈强弥漫性表达，嗜铬粒蛋白A呈局灶性表达。这是关于转移性副脊索瘤病例的第五份文献报道。这些肿瘤很少表现出如此侵袭性的行为；此外，这些肿瘤对嗜铬粒蛋白A的免疫反应性出乎意料。本文讨论了所谓的副脊索瘤与软组织肌上皮瘤/混合瘤之间的关系，包括最近定义的儿童肌上皮癌。
