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第171课 病例探析(023)—骶椎脊索瘤

 zskyteacher 2019-08-16

上期试题:下列哪些肿瘤最容易发生骨转移,常被称为“亲骨性肿瘤”(多选):ACE

A.乳腺癌  

B.肝癌

C.前列腺癌  

D.甲状腺癌

E.肺癌

患者,男,61岁,腰骶疼痛3个月余,近1周加重。

CT平扫见骶椎骨质破坏,椎体前方可见一类圆形软组织肿块,边缘可见斑点状钙化。

MRI示骶1-5椎体骨质破坏,以骶3椎体破坏为著,肿块呈不规则形,于T1WI像上呈低信号,于T2WI及T2WI抑脂像上呈明显高信号,肿瘤内部可见分房状改变,肿瘤向前方生长,形成一巨大软组织肿块,其上缘与骶椎呈锐角相连,肿块向后方椎管内突起,继发椎管狭窄。

      正常情况下脊索是人体脊柱的原基,在胚胎2个月后即消失。脊索瘤起源于胚胎残存的脊索组织,由分叶状排列的含有空泡的囊泡性细胞和黏液样细胞组成,是一种少见的恶性肿瘤,占原发性骨肿瘤的2%-4%,占恶性骨肿瘤的1%。可发生于任何年龄,以40-50岁男性多见。

      The notochord is human body normal spinal primordium, disappeared in 2 months after embryo. Chordoma originated from embryonic survival of notochord organization by lobulated arranged vesicles containing cavity of sexual cells and myxoid cells, is a rare malignant tumor, accounts for 2% to 4% of primary bone tumors, 1% of malignant bone tumors. Can happen at any age, with 40-50 men. 

      脊索瘤生长缓慢,早期症状轻微,常在肿瘤累及多个椎体或肿块较大后才被发现。脊柱脊索瘤最常见部位是骶尾部,其次是枢椎。骶尾部脊索瘤多见于第2-5骶骨,肿瘤常累及相邻的2个椎体,骶1椎体单独受累者少见。脊柱脊索瘤早期表现为椎体的轻度膨胀性骨质破坏,边界清楚,随着病变的缓慢发展,肿瘤更易突破椎体前缘骨皮质并形成较大的圆形或类圆形肿块,向前推压相邻结构。少数病例伴有向后推压硬膜囊和脊髓,继发椎管狭窄,仅向椎管内侵犯的肿瘤少见。在正中矢状面上,骶尾部脊索瘤形成的椎体前方巨大肿块上缘常与椎体呈锐角相连,这可能是肿瘤突破骶椎骨皮质后,生长速度加快所致。肿瘤晚期可发生淋巴转移和血行转移。

      Chordoma growth is slow, early symptom is slight, often in tumor is involved in multiple vertebral body or lump is bigger hind just be discovered. The most common part of spinal chordoma is the sacrosacral tail, followed by the armature vertebra. The sacral caudal chordoma is often seen in the 2nd -5 sacrum, and the tumor is often involved in two adjacent vertebral bodies, and the sacral 1 vertebral body alone is rare. Early spinal chordoma characterized by mild dilatability of vertebral body bone destruction, border and clear, with the slow development of lesions, tumors are more likely to break through the fanterior edge bone cortex and form a larger round or class round mass, push forward the adjacent structure. A small number of cases are associated with a retro-thrust epidural sac and spinal cord, and secondary spinal stenosis, which is rarely seen in the tumor of the spinal canal. In the median sagittal plane, the formation of the rear of the sacral chordoma vertebral body in front of the huge lump on margin often connected to the vertebral body in acute Angle, it could be a tumor after breakthrough sacral vertebral cortex, grow faster. Lymphatic metastasis and metastasis can occur in the late stage of the tumor. 

      脊柱脊索瘤的信号在T1WI像上呈低信号,在T2WI及抑脂像上呈高信号,其内信号不均匀。肿瘤内常合并钙化,钙化灶于各个序列上呈等或低信号。当肿瘤内合并出血时,表现为不均匀的短T1、长T2信号,如果合并坏死和囊变,则肿瘤内可见更长T1、更长T2信号影。增强扫描时,脊索瘤常呈不均匀强化,其内可见多个斑点状、斑片状等低信号影,病灶的持续强化时间较长。

       The signal of spinal chordoma is low signal on T1WI and high signal on T2WI and lipostatic image, and its internal signal is not uniform. The tumor is often calcified and calcified in various sequences of the same or low signal. When the tumor is combined with hemorrhage, it is shown as an uneven short T1 and long T2 signal. If the necrosis and cystic changes are combined, the tumor can be seen with longer T1 and longer T2 signal shadows. In the enhancement of scanning, chordoma is often unevenly strengthened, with many spots, patches and other low-signal shadows, and the continuous strengthening time of the lesions is longer. 

       本例应于单发性骨转移瘤、脊椎骨巨细胞瘤、脊椎嗜酸性肉芽肿鉴别。单发性骨转移瘤大多无膨胀性改变,常向后侵及两侧椎弓根,在椎体周围形成软组织肿块,很少单独形成椎体前方较大软组织肿块,如果能查找到原发灶,可明确诊断。脊椎骨巨细胞瘤以骶1-3椎体多见,好发年龄为20-40岁,呈偏一侧 的膨胀性生长,破坏区呈‘皂泡样’改变,内可见粗大的骨性间隔,钙化少见,边缘无硬化带。脊椎嗜酸性肉芽肿常见于儿童、青少年,椎体破坏呈‘硬币样’压缩性骨折,可伴椎体周围厚薄均匀的软组织肿块。

      This case should be identified by single - hair metastatic tumor, vertebral giant cell tumor, and spine eosinophilic granuloma. Single bone metastases are no expansion, often abuse and back on both sides of the pedicle, around vertebral body mass lesion formation, rarely separate form vertebral body in front of the large soft tissue mass, if we can find to the original site, can be a definitive diagnosis. Sacral spinal giant cell tumors in 1-3 vertebral bodies, for the age of 20 to 40 years old, the skew the expansion growth, on one side of the destroyed area show 'soap bubble samples' change, bulky osseous intervals, can be seen in the calcification is rare, no hardening zone. The spinal eosinophilic granuloma is common in children and adolescents, and vertebral body damage is a 'coin like' compressibility fracture, but it can be accompanied by a thin uniform soft tissue mass around the vertebral body. 

本期试题:颅内脊索瘤好发部位为?

A.蝶鞍部

B.桥小脑角区

C.颅前窝

D.颅底枕软骨结合处

E.颈静脉孔区

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