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There were 36 males and 40 females,with the average age of 47.7 years.36 patients presented with sciatica and perineal numbness,11 patients had painless mass and the other 20 were identified due to other causes.Among 67 benign neurogenic tumors,54 were originated from S1-S3 nerves,3 from S4-S5 nerves and 11 from the presacral space without caudal involvement.Giant cell tumor was found in 60 patients,which included 24 males and 36 females,with an average age of 32 years.Upper sacrum(S1-S2) was involved firstly.There were 56 patients(average,37.7 years) diagnosed as other primary benign bone tumors,which included 24 cases of teratomas,epidermoid cyst or dermoid cyst.Other malignant tumors,including chondrosarcoma (17),Ewing′s sarcoma/PNET(14),multiple myeloma(12),lymphoma(6) and osteosarcoma(6) were found in 64 patients.

其中脊索瘤95例,男62例,女33例,平均年龄55.7岁,骶尾区疼痛是主要症状(82例),半数以上患者伴有坐骨神经痛,肿瘤多先累及低位骶骨(S3~S5);神经源性肿瘤76例,男36例,女40例,平均年龄47.7岁,神经纤维瘤43例,神经鞘瘤24例,恶性神经鞘瘤9例,主诉多为坐骨神经痛、会阴部麻木(36例)和无痛性包块(11例),20例患者为查体或其他原因检查时发现,67例骶骨良性神经源性肿瘤起源于S3以上神经者54例,起源于S3以下神经者3例,发生于骶前未累及骶管者10例;骨巨细胞瘤60例,男24例,女36例,平均年龄32岁,腰骶尾部不适(37例)、坐骨神经痛(21例)是主要的临床表现,肿瘤多先累及上位骶骨(S1~S2);骶骨其他原发良性肿瘤及瘤样病变56例,包括畸胎瘤、皮样囊肿、表皮样囊肿共24例;其他原发恶性肿瘤64例,包括软骨肉瘤17例,尤文肉瘤14例,多发性骨髓瘤12例,成骨肉瘤6例等。

Our findings demonstrate that in conjunction with clinical and radiographic findings, immunohistochemical evaluation with a panel of D2-40, EMA, brachyury, and GFAP is most useful in distinguishing chordoid meningioma from chordoid glioma, skeletal myxoid chondrosarcoma, extraskeletal myxoid chondrosarcoma, chordoma, low-grade chondrosarcoma, and enchondroma. A lack of strong, diffuse S100 reactivity may also be useful in excluding chordoid meningioma.

我们的结果表明,结合临床和影像学资料,D2-40、EMA、brachyury和GFAP这样一个免疫标记组合就能很好地鉴别脊索样脑膜瘤和脊索样胶质瘤、骨的黏液样软骨肉瘤、骨外的黏液样软骨肉瘤、脊索瘤、低级别软骨肉瘤、内生性软骨瘤。S100染色不强烈、不弥漫也有助于排除脊索样脑膜瘤的诊断。

EMA was the most effective antibody for differentiating chordoid meningioma from skeletal myxoid chondrosarcoma, low-grade chondrosarcoma, and enchondroma, whereas D2-40 was the most effective antibody for differentiating chordoid meningioma from extraskeletal myxoid chondrosarcoma and chordoma.

EMA是鉴别脊索样脑膜瘤和骨的黏液样软骨肉瘤、低级别软骨肉瘤、内生性软骨瘤最有效的抗体,而D2-40则是鉴别脊索样脑膜瘤和骨外黏液样软骨肉瘤、脊索瘤最有效的抗体。

Disc cell mainly consist of chondrocytes, PTHrP-Ihh negative feedback mechanism can modulate differentiation and maturation of chondrcytes during endocartilage ossification in epiphysis of long shaft bone, but its effect on vertebral development is still unknown.

椎间盘细胞主要由软骨细胞样细胞组成,PTHrP和Ihh形成的负反馈机制在长管骨软骨内骨化过程中对软骨细胞分化和成熟具有调节作用,在椎体和椎间盘的发育过程中的作用仍未阐明。

Staining extent and intensity were evaluated semiquantitatively and mean values for each parameter were calculated. Immunostaining with D2-40 showed positivity in 100% of skeletal myxoid chondrosarcomas, 96% of enchondromas, 95% of low-grade chondrosarcomas, 80% of chordoid meningiomas, and 75% of chordoid gliomas. Staining with S100 demonstrated diffuse, strong positivity in all (100%) chordoid gliomas, skeletal myxoid chondrosarcomas, low-grade chondrosarcomas, and enchondromas, 94% of chordomas, and 81% of extraskeletal myxoid chondrosarcomas, with focal, moderate staining in 40% of chordoid meningiomas.

我们半定量地评估了这些免疫染色的广度和强度,并且计算了它们各自的平均值。D2-40阳性表达于100%例骨的黏液样软骨肉瘤、96%例内生性软骨瘤、95%例低级别软骨肉瘤、80%例脊索样脑膜瘤和75%例脊索样胶质瘤。S100染色弥漫且强烈地表达于所有的(100%)脊索样胶质瘤、骨的黏液样软骨肉瘤、低级别软骨肉瘤和内生性软骨瘤,94%例脊索瘤,81%例骨外黏液样软骨肉瘤,还有,局灶性、中度表达于40%例黏液样脑膜瘤。

In this study, immunohistochemical staining was performed with antibodies against D2-40, S100, pankeratin, epithelial membrane antigen, brachyury, and glial fibrillary acidic protein in 4 cases of chordoid glioma, 6 skeletal myxoid chondrosarcomas, 10 chordoid meningiomas, 16 extraskeletal myxoid chondrosarcoma, 18 chordomas, 22 low-grade chondrosarcomas, and 27 enchondromas.

本研究中,我们给4例脊索样胶质瘤、6例骨的黏液样软骨肉瘤、10脊索样脑膜瘤、16例骨外黏液样软骨肉瘤、18例脊索瘤、22例低级别软骨肉瘤和27例内生性软骨瘤做了D2-40、S100、pankeratin、上皮膜抗原、brachyury和胶质纤维酸性蛋白的免疫组化染色。

Often is the result of a lack of vitamin D, costochondral hyperplasia, all rib cartilage hyperplasia even bead-like effect may often oppressed the lungs, so that children with poor ventilation, easy to suffer from bronchitis, pneumonia.

常有串珠肋,是由于缺乏维生素D,肋软骨增生,各个肋骨的软骨增生连起似串珠样,常压迫肺脏,使小儿通气不畅,容易患气管炎,肺炎。

Results Among the three groups,the children's rib cartilage had the most blood vessels,the most chondrocytes,well-distributed stain of matrixes,and the type Ⅱ collagen was expressed actively and highest in photedensity.The rib cartilage of teenager group had less blood vessels,unhomogeny distributed stain of matrixes,the enlarged and separated cartilage lacunas.The rib cartilage in adult group showed the least blood vessels,the least chondrocytes.the hyalinization of perichondium,the most deposition of calcium salt,and the type II collagen was expressed at the lowest level in photodensity.

结果 儿童组肋软骨膜血管最丰富,软骨基质染色均匀,软骨细胞数目最多,Ⅱ型胶原蛋白表达最活跃,平均积分光密度值最高;青少年组软骨膜内血管减少,软骨基质染色出现明显的不均质状,软骨陷窝体积变大,并呈分隔状,陷窝内软骨细胞数目减少,II型胶原蛋白表达较儿童组减弱;成人组软骨膜血管、细胞成分明显减少,软骨膜内的纤维成分明显玻璃样变,钙盐沉积较青少年组时明显增多,Ⅱ型胶原蛋白表达较青少年组减弱。

To be qualified as an EOS, the tumor must (1) arise in the soft tissue and not be attached to bone or periosteum,(2) have a uniform sarcomatous pattern ( to exclude the possibility of a mixed malignant mesenchymal tumor),(3) produce osteoid and/or cartilage matrix.

本病的诊断标准是(1)发生于软组织而与骨及骨膜部不相连;(2)具有一致的骨肉瘤表象,以排除混合性恶性间叶组织肿瘤;(3)可产生骨质或软骨样基质。

7Bone shell or shell-liked calcification in the rim of soft-tissue masses: arc-liked high density lied in the rim of soft-tissue masses , concave surface face to the bone leisions.

CT检查显示软组织肿胀0度2例,I度19例,11度16例,111度17例。6例软组织内出现脓肿样囊腔。1例显示软组织内气体。1例显示脂液平面。1例显示窦道。5例显示软组织肿块。8例示软组织内难以定性的钙质样高密度影。54例CT检查均未见软组织肿块边缘残留骨壳或壳样钙化和软组织肿块内肿瘤骨或瘤软骨钙化。

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