第162课 病例探析（020）—腓骨韧带样纤维瘤

上期试题：水杨酸类药物可缓解疼痛的骨瘤是A

A.骨样肿瘤

B.软骨肿瘤

C.骨肉瘤

D.骨瘤

E.骨软骨瘤

患者，男，20岁，左小腿肿胀、疼痛2年，近期疼痛加重

查体：左小腿近端增粗，局部无红肿，皮温不高，左膝及左足屈伸活动可。

↑CT平扫：

左腓骨中上段呈明显膨胀性骨质破坏，骨皮质变薄呈蛋壳样，局部骨皮质中断、不连续，边缘可见残留的粗大骨嵴，其内密度不均匀。

↑CT平扫 、冠状位T1WI

↑冠状位T2WI、横轴位T2WI抑脂像

左腓骨中上段明显膨胀性骨质破坏，呈混杂T1、混杂T2信号，T2WI抑脂像上呈中等及略高信号，边缘见蛋壳样低信号，周围软组织肿胀。

            骨韧带样纤维瘤亦称骨促结缔组织增生性纤维瘤、骨硬纤维瘤，是一种以肿瘤细胞产生丰富胶原纤维为特征的良性肿瘤，具有侵袭生长、易复发的特点。好发于30岁以前的青少年，无明显性别差异，好发于长骨，尤其是骨骺未闭前的干骺端，并向骨干生长，较少累及骨骺，常见发病部位为股骨、胫骨、下颌骨及骨盆等。按病变部位分为两种类型，即中央型和骨旁型，各型之间的影像学表现差异较大，确诊依靠病理学检查。基本影像学表现：以囊状膨胀性骨质改变为主，内见残留粗大骨嵴，骨皮质变薄，边缘清晰，可伴有硬化边，少数为溶骨性骨质破坏，骨皮质消失，肿瘤突破骨皮质向周围形成边缘清晰的软组织肿块，其内无钙化或骨化，MRI检查T1WI像及T2WI像上，病变区信号与肌肉相等或略低。特征性改变为‘树根’或‘胡须’样肿瘤性骨小梁形成。

           Bone ligaments sample fibroma also called bone to promote connective tissue proliferative fibroma, desmoid tumors of bone, is a kind of characterized by rich tumor cells to produce collagen fibers of benign tumors, has the characteristics of the invasive growth, easy to relapse.Good hair at the age of 30 teenagers, no significant gender differences, good hair at long bone, especially the stem epiphyseal end, prior to close of epiphyseal growth and to the backbone, less involvement epiphyseal, common parts of the femur, tibia, mandible and pelvis, etc.According to the lesion site was divided into two types, namely the central and near the bone, the imaging findings of difference between each model is bigger, diagnosis depends on pathological examination.Basic findings: give priority to with cystic expansion bone change, see residues in bulky bone crest, cortical thinning of bone, the edge is clear, may be accompanied by hardening edge, a few of rock-salt osseous bone destruction, bone cortex disappear, breaks through the bone cortex to form around tumor edge clear soft tissue mass of them without calcification or ossification, MRI T1WI and T2WI like like, lesion area equal or slightly low signal and muscles.Characteristic change to 'root' or 'whiskers' tumor bone trabecular formation.

本例应与多房性骨囊肿、动脉瘤样骨囊肿、软骨黏液样纤维瘤鉴别。

This example should be identified with multi-atrial bone cysts, aneurysmal bone cysts and chondroid fibroid tumors。

       多房性骨囊肿最常见于20岁以下青少年和儿童，呈圆形或椭圆形膨胀性透亮区，多数位于干骺或骨干髓腔，长轴与骨长轴一致，横径一般不超过骨骺板宽度，皮质膨胀变薄，边缘可见薄层骨质硬化环。

         Multilocular bone cyst is most common in adolescents and children under the age of 20, assumes the circular or elliptic dilatability is bright area, many of which are in dry epiphyseal or backbone medullary cavity, long axis is consistent with the long axis of the bone, the transverse diameter is generally not more than the epiphyseal plate width, cortical thinning, edge visible thin layer of bone sclerosis ring.

      动脉瘤样骨囊肿发病年龄多在10~30岁，常累及长管状骨的干骺端，病变多呈膨胀性、溶骨性骨质破坏，偏心性生长，外观呈蜂窝状或皂泡状，液液平面是其特异性征象。

      Aneurysm sample bone cyst onset age in 10 ~ 30 more, often involving the stem epiphyseal end of the long tubular bones, lesions were swelling and dissolving osseous bone destruction, more wide mind growth, appearance is honeycombed or soap bubbly, liquid-liquid plane is its specific signs.

      软骨黏液样纤维瘤发病年龄多在10~30岁，好发于下肢长管骨，其中以胫骨上端多见，基本影像学特征为偏心性、囊状、膨胀性骨质破坏，骨皮质膨胀变薄，并形成较厚的骨壳，囊壁可见伸向囊内的粗大骨嵴，病灶内可见钙化灶。

       Cartilage myxoid fibroma onset age in 10 ~ 30 years old, good hair at long tube bone of lower limb, of which upper tibia, radiographic features of the basic mind bone destruction, cystic, swelling, bone cortex thins, and form a thick bone and shell, inside the capsule wall visible to bulky bone crest, calcifications can be seen in the lesion.

↑另外一例腓骨韧带样纤维瘤的影像表现

本期试题：男性，18岁患者，左上臂近端疼痛、肿胀1个月。就诊时X线片显示：左肱骨上段膨胀性囊状透亮区，边界清，内有骨性间隔将囊腔分成蜂窝状。最可能的诊断是？

A.骨囊肿

B.骨纤维异样增殖症

C.动脉瘤样骨囊肿

D.内生性软骨瘤

E.骨肉瘤