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第252课 (骨肌系统)病例探析(073)-骨软骨瘤

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骨软骨瘤

临床与病理

本病很常见,好发于10-30岁,男性多于女性。本病多见于四肢长骨,尤其好发于长骨干骺端部位,以股骨下端和胫骨上端最常见,约占50%。临床上早期一般无明显症状,仅在病变的局部可扪及一硬结。肿瘤增大时可有轻度压痛和局部畸形,近关节的肿瘤可引起活动障碍,或可压迫邻近的神经而引起相应的症状。若肿瘤突然长大或生长迅速,应考虑有恶变的可能。肿瘤通常位于干骺端的一侧骨皮质,向骨表面生长,可单发和多发,单发多见,多发性骨软骨瘤病为一种先天性骨骼发育异常,是由双亲传递的常染色体显性遗传病。

This disease is very common. It usually occurs between 10 and 30 years old, and more men than women. This disease sees in limb long bone more, good hair is in especially long diaphysis end place, with femur inferior end and tibia top end most common, hold about 50%. Clinical early general no obvious symptoms, only local palpable lesions and a scleroma. When tumour increases, can have gentle tenderness and local deformity, the tumour near joint can cause activity obstacle, or can compress adjacent nerve and cause corresponding symptom. If the tumor grows suddenly or rapidly, the possibility of malignant transformation should be considered. The tumor is usually located in the bone cortex on one side of the epiphyseal end and grows to the bone surface. It can occur in single or multiple cases. Multiple osteochondromatosis is a congenital skeletal dysplasia and an autosomal dominant genetic disease transmitted by both parents.

病理:骨软骨瘤又名骨软骨性外生骨疣,是指在骨的表面覆以软骨帽的骨性突出物。骨软骨瘤可发生于任何软骨内化骨的骨,长骨干骺端是其好发部位。骨软骨瘤是最常见的骨肿瘤,占骨良性肿瘤的迫近 1/3,为最常见的良性骨肿瘤。可分为单发和多发性骨软骨瘤,绝大多数为单发,仅少数为多发。多发性骨软骨瘤病又称遗传性多发性外生骨疣,为一种先天性骨骼发育异常,是由双亲传递的常染色体显性遗传病。骨软骨瘤是一个带蒂的或无柄的骨性隆起,可分为宽基型与带蒂型两种,从骨表面向外隆起,表面呈半球状、菜花状或息肉状。肿瘤由骨性基底、软骨帽、纤维包膜三部分组成。骨性基底为肿瘤的主体,常占肿瘤的大部分,其可宽可窄,内为骨小梁和骨髓,骨小梁间多为纤维组织,有较丰富的毛细血管网,外被薄层骨皮质,两者均分别与母体骨的相应部分连续。软骨帽位于骨性基底的顶部,由灰白略带蓝色的透明软骨组成,其厚度一般随年龄增大而减退,至成年可完全骨化。纤维包膜即软骨膜,为一薄层纤维组织,和相邻骨膜相连。在骨软骨瘤的顶端,可有一个继发性滑囊形成,滑囊的内面可有滑膜覆盖,囊腔内有时可以有游离体。

Osteochondroma, also known as osteochondral exophytic osteoperiota, is a bony protrusion covering the surface of bone with a cartilaginous cap. Osteochondroma can occur in the bone of any cartilage internalized bone. Osteochondroma is the most common benign bone tumor, accounting for nearly 1/3 of all benign bone tumors. Can be divided into single and multiple osteochondromas, the vast majority of single, only a few of multiple. Multiple osteochondromatosis, also known as hereditary multiple ectoma, is an autosomal dominant inherited disease transmitted by both parents. Osteochondroma is a pedicled or sessile osteophytic protuberant, which can be divided into broad basal type and pedicled type. It is protuberant from the surface of bone and appears as hemispherical, cauliflower or polypoid surface. The tumor consists of three parts: bone base, cartilage cap and fibrous capsule. The bony base is the main body of the tumor, which often accounts for the majority of the tumor. It can be wide or narrow, with trabecular bone and bone marrow inside, and fibrous tissue between trabecular bone and abundant capillary network, and thin layer of bone cortex outside, both of which are respectively continuous with the corresponding part of the maternal bone. The cartilaginous cap is located at the top of the bony base and consists of pale and bluish hyaline cartilage. The thickness of the hyaline cartilage generally decreases with age and can be completely ossified in adulthood. The fibrous capsule, or perichondrium, is a thin layer of fibrous tissue attached to an adjacent periosteum. At the tip of an osteochondroma, a secondary synovial bursa may be formed. The inner surface of the bursa may be covered by synovium, and the lumen may sometimes contain free bodies.

CT上瘤体主要包括骨性基底和软骨盖帽两部分。前者的骨皮质和骨松质均与母体骨相延续,发生于长管状骨者多背离关节生长,其内可见骨小梁,且与母骨的小梁相延续。基底部顶端略为膨大,呈菜花状或丘状隆起。基底部顶缘为不规则的致密线。骨性基底表面有软骨覆盖,软骨帽边缘多光整,呈软组织密度影,其内可见点状或环形钙化或骨化影。增强扫描瘤灶无明显强化。

The upper tumor body in CT mainly consists of two parts: bony basement and cartilaginous cap. The cortical and cancellous bone of the former bone extended with the parent bone, and the long tubular bone mostly deviated from the joint growth, in which trabecular bone could be seen, and the trabecular bone continued with the mother bone. Basal tip slightly expanded, cauliflower or hump - like uplift. The top edge of the basement is an irregular dense line. The bony basal surface was covered with cartilage, and the edges of the cartilage cap were smooth and smooth, showing soft tissue density shadow, in which there were dotted or annular calcification or ossification shadow. No obvious enhancement was found in the enhanced scan.

MR:骨性基底部的信号特点与母体骨相同;软骨帽在T1WI上呈低信号影,脂肪抑制T2WI上为明显的高信号,其信号特点与关节透明软骨信号很相似。由于 MRI 能清楚显示软骨帽,因此,有助于评估骨软骨瘤是否恶变,若软骨帽厚度大于2cm,则提示为恶变。

MR: the signal characteristics of bony basement were the same as those of maternal bone. The cartilage cap showed a low signal shadow on T1WI, while the fat inhibition on T2WI showed an obvious high signal, which was similar to the hyaline cartilage signal of joints. Since MRI can clearly show the cartilaginous cap, it is helpful to assess whether osteochondroma is malignant. If the thickness of the cartilaginous cap is greater than 2cm, it is indicated as malignant.

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