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软骨肉瘤的形态学分型:94例病例研究

Morphological typing of chondrosarcoma: a study of 94 cases.

作者信息

Welkerling H, Dreyer T, Delling G

机构信息

Department of Bone Pathology, University of Hamburg, Federal Republic of Germany.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1991;418(5):419-25. doi: 10.1007/BF01605928.

Abstract

Ninety-four chondrosarcomas of the Hamburg Bone Tumour Registry were reviewed in a retrospective study. The purpose of this study was to examine the morphological characteristics of different types of chondrosarcomas and to describe distinctive features of location, the age distribution and the male to female ratio. Central chondrosarcomas can be divided into classical chondrosarcomas, dedifferentiated chondrosarcomas, mesenchymal chondrosarcomas and clear-cell chondrosarcomas. Five periosteal chondrosarcomas were represented. Classical chondrosarcomas and clear-cell chondrosarcomas show a significant predominance of males; no sex predilection was seen in dedifferentiated and mesenchymal chondrosarcomas. Nearly 60% of classical and mesenchymal chondrosarcomas occur in the trunk. Eighty-five percent of dedifferentiated chondrosarcomas are located in the long bones of the limbs. Clear-cell chondrosarcomas arise in the proximal part of the femur. There is a marked predilection for mesenchymal chondrosarcomas in the second and third decades of life. The average age of patients with classical chondrosarcomas was 54 years, but clear-cell chondrosarcomas occur 10 years earlier and dedifferentiated chondrosarcomas 10 years later. Characteristically, classical chondrosarcomas produce a pure chondroid matrix with variable differentiation of tumour chondrocytes. The most important histological feature of the defifferentiated chondrosarcoma is the close association of two different cellular components. One of these consists of cartilage, which is generally well differentiated. In most of our cases the second component showed features of osteosarcoma (50%). Mesenchymal chondrosarcoma is characterized by concentric infiltration of cartilage islands by small tumour cells. Clear-cell chondrosarcomas show regions of cartilaginous tumour and areas of closely packed, glycogen-rich, large tumour cells with distinct boundaries. Osteoid formation and multinucleated giant cells are present in clear-cell areas. Knowledge of this group of tumours is indispensable for correct histological diagnosis and typing and is important in the design of surgical therapy and the prediction of biological behaviour.

摘要

对汉堡骨肿瘤登记处的94例软骨肉瘤进行了回顾性研究。本研究的目的是检查不同类型软骨肉瘤的形态特征,并描述其位置、年龄分布及男女比例的显著特征。中央型软骨肉瘤可分为经典型软骨肉瘤、去分化软骨肉瘤、间叶性软骨肉瘤和透明细胞软骨肉瘤。有5例为骨膜软骨肉瘤。经典型软骨肉瘤和透明细胞软骨肉瘤男性明显居多;去分化和间叶性软骨肉瘤未见性别偏好。近60%的经典型和间叶性软骨肉瘤发生于躯干。85%的去分化软骨肉瘤位于四肢长骨。透明细胞软骨肉瘤发生于股骨近端。间叶性软骨肉瘤在第二和第三个十年有明显的发病偏好。经典型软骨肉瘤患者的平均年龄为54岁,但透明细胞软骨肉瘤发病早10年,去分化软骨肉瘤发病晚10年。典型的是,经典型软骨肉瘤产生纯软骨样基质,肿瘤软骨细胞有不同程度的分化。去分化软骨肉瘤最重要的组织学特征是两种不同细胞成分紧密相连。其中一种由软骨组成,通常分化良好。在我们的大多数病例中,第二种成分显示骨肉瘤特征(50%)。间叶性软骨肉瘤的特征是小肿瘤细胞呈同心性浸润软骨岛。透明细胞软骨肉瘤表现为软骨性肿瘤区域和边界清晰的紧密排列、富含糖原的大肿瘤细胞区域。透明细胞区域可见类骨形成和多核巨细胞。了解这组肿瘤对于正确的组织学诊断和分型必不可少,并且在手术治疗设计和生物学行为预测方面很重要。

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