Department of Anatomic Pathology, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Skeletal Radiol. 2012 Dec;41(12):1641-4. doi: 10.1007/s00256-012-1493-3. Epub 2012 Aug 11.
We report a unique case of mediastinal paravertebral chordoma without bone destruction in a 47-year-old Japanese woman. She was admitted to hospital after a tumor was incidentally detected on a chest radiograph. The tumor was located in the paravertebral region of the mediastinum and did not show any destruction of the thoracic vertebra radiologically. The tumor was clinically diagnosed as a benign neurogenic tumor and the tumor was easily removed surgically. Microscopically, the tumor mainly consisted of tumor cells with extensively vacuolated cytoplasm, arranged in cord- and nest-like fashion against a myxoid matrix background. Immunohistochemically, the tumor cells showed diffuse positivity for pancytokeratin (AE1/AE3) and vimentin. The tumor cell nuclei were positive for brachyury, which is a key transcription factor of notochordal development. These results confirmed the tumor to be an extraosseous chordoma in the paravertebral mediastinal region, which is an extremely rare location for a chordoma.
我们报告一例 47 岁日本女性无骨破坏的纵隔椎旁脊索瘤。患者因胸片偶然发现肿瘤而入院。肿瘤位于纵隔椎旁区,影像学上未见任何胸椎破坏。临床诊断为良性神经源性肿瘤,肿瘤易于手术切除。显微镜下,肿瘤主要由具有广泛空泡化细胞质的肿瘤细胞组成,呈索状和巢状排列,背景为黏液样基质。免疫组化染色显示肿瘤细胞弥漫性表达细胞角蛋白(AE1/AE3)和波形蛋白。肿瘤细胞核阳性表达 brachyury,这是脊索发育的关键转录因子。这些结果证实该肿瘤为位于纵隔椎旁的骨外脊索瘤,这是脊索瘤极为罕见的部位。