背景:脊索瘤,一种罕见的由脊索组织引起的恶性肿瘤,通常发生在脊柱轴。只有少数已发表的原发性肺脊索瘤的报道。在这里,我们介绍了一例原发性肺脊索瘤,并讨论了诊断罕见脊索瘤的重要考虑因素。
方法:我们报告一例39岁男性原发性肺脊索瘤,有睾丸混合生殖细胞卵黄囊肿瘤和畸胎瘤病史。计算机断层扫描显示左下叶缓慢生长的实性病变。我们对可疑生殖细胞肿瘤肺转移进行了楔形切除术。组织学上,大的圆形或卵圆形细胞与嗜酸性细胞的细胞质被大的细胞包围,颗粒,轻度嗜酸性细胞浆。肿瘤细胞为生唾液。免疫组织化学检查对短尾畸形呈阳性,S-100蛋白,上皮膜抗原,波形蛋白,和细胞角蛋白AE1/AE3,提示肺脊索瘤。睾丸混合生殖细胞肿瘤的重新检查未发现脊索元素。尽管某些区域的短尾染色呈阳性,苏木精和伊红(HE)染色未显示脊索瘤的典型形态特征。肺肿瘤的互补荧光原位杂交(FISH)证实不存在同染色体12p和12p扩增。因此,确定了原发性肺脊索瘤的最终诊断.
结论:在有睾丸混合生殖细胞肿瘤病史的患者中,肺和睾丸肿瘤的HE和Brachyury染色的组织形态学比较,使用FISH分析肺肿瘤中的12p和12p扩增对于诊断罕见肺脊索瘤至关重要。
BACKGROUND: Chordoma, a rare malignant tumor arising from notochordal tissue, usually occurs along the spinal axis. Only a few published
reports of primary lung chordomas exist. Herein, we present a
case of primary lung chordoma and discuss important considerations for diagnosing rare chordomas.
METHODS: We report a
case of primary lung chordoma in a 39-year-old male with a history of testicular mixed germ-cell tumor of yolk sac and teratoma. Computed tomography revealed slow-growing solid lesions in the left lower lobe. We performed wedge resection for suspected germ-cell tumor lung metastasis. Histologically, large round or oval cells with eosinophilic cytoplasm were surrounded by large cells with granular, lightly eosinophilic cytoplasm. Tumor cells were physaliphorous. Immunohistochemistry was positive for
brachyury, S-100 protein, epithelial membrane antigen, vimentin, and cytokeratin AE1/AE3, suggesting pulmonary chordoma. Re-examination of the testicular mixed germ-cell tumor revealed no notochordal elements. Although some areas were positive for
brachyury staining, hematoxylin and eosin (HE) staining did not show morphological features typical of chordoma. Complementary fluorescence in situ hybridization (FISH) of the lung tumor confirmed the absence of isochromosome 12p and 12p amplification. Thus, a final diagnosis of primary lung chordoma was established.
CONCLUSIONS: In patients with a history of testicular mixed germ cell tumors, comparison of histomorphology using HE and Brachyury staining of lung and testicular tumors, and analyzing isochromosome 12p and 12p amplification in lung tumors using FISH is pivotal for the diagnosis of rare lung chordomas.