本文报告13例罕见的脊索瘤,计骶尾部发生8例,蝶枕2例,胸,腰椎及鼻咽部各1例。男(6例)、女(7例)性发生率相似,年龄18～75岁,平均48岁。此瘤生长缓慢,恶性度低,早期无明显临床表现,随后出现局部疼痛、肿块及其它症状。X片见溶骨性变,有时伴有点状钙化。肉眼观,呈球形或不规则形肿块,质地硬或软,肿瘤可巨大(如例10,肿瘤50×50×40cm,重约30斤)。切面示分叶状结构,呈灰白或粉红色,半透明、胶冻状。镜下,瘤由圆形或多边形细胞组成,常呈腺泡状或索状排列。典型瘤细胞可含有许多粘液空泡(特称之为囊泡状细胞)。有时瘤问或间质内含有大量粘液样物质,甚至形成粘液湖,瘤细胞呈岛状散在其间。此外,对脊索瘤与软骨瘤,软骨肉瘤,转移性粘液癌,软组织脂肪肉瘤和脊索样瘤等作了简要的讨论。 In this paper, 13 cases of rare chordomas were reported, including 8 cases of orbital tail, 2 cases of butterfly occipital, and 1 case of thoracic, lumbar and nasopharynx. Male (6 cases) and female (7 cases) were similar in sexual activity, aged 18 to 75 years, with an average age of 48 years. This tumor has a slow growth, a low degree of malignancy, no obvious clinical manifestations at the early stage, and subsequent local pain, lumps, and other symptoms. X tablets see osteolytic change, sometimes with a bit like calcification. From the naked eye, it is spherical or irregularly shaped, and the texture is hard or soft. The tumor can be huge (as in Example 10, the tumor is 50 x 50 x 40 cm, weighing about 30 pounds). Sections show lobulated structures, gray or pink, translucent, jelly-like. Microscopically, the tumor consists of round or polygonal cells, often in alveolar or cord-like arrangements. A typical tumor cell can contain many mucus vacuoles (specially referred to as vesicular cells). Sometimes the tumor or the interstitium contains a lot of mucous-like substances, and even forms a mucus lake, where the tumor cells are scattered in islands. In addition, brief discussions were made on chordoma and chondroma, chondrosarcoma, metastatic mucinous carcinoma, soft tissue liposarcoma, and chordoid tumor.