脊柱朗格汉斯细胞组织细胞增生症

Bang

9岁男孩，下腰痛。

增强CT横断位骨窗（图A）显示L2椎体后缘溶骨性病灶，边界不清，局部骨皮质不连续。横断位T1WI(图B)病灶呈稍高信号，椎体后缘骨皮质不联系，病灶侵犯硬脊膜及右侧椎旁间隙。STIR序列（图C）病灶呈弥漫性高信号。增强扫描横断位T1WI(图D)及矢状位FS T1WI(图E)示病灶明显强化。

Langerhans Cell Histiocytosis (LCH) of the Spine

脊柱朗格汉斯细胞组织细胞增生症

背景

LCH comprises a rare group of disorders with a wide range of clinical presentations.

LCH是一组罕见的病变，临床表现多种多样。

It is characterized by abnormal accumulation of histiocytes in different tissues, but the etiology remains unknown

典型表现为不同组织内组织细胞的异常积聚，但病因尚不完全明确。

LCH is more common in children and adolescents.

LCH多见于儿童和青少年。

Bone is the most common site affected, with a predilection for the axial squeleton.

骨骼是LCH的好发部位之一，尤其是中轴骨更易受累。

临床表现

Pain, neurologic deficits, restricted motion, deformity. Some patients may be asymptomatic.

主要临床表现为疼痛、神经功能缺损、运动受限、畸形等。也可有部分患者无明显症状。

影像诊断要点

Solitary or multiple lytic lesions, round or oval, with poor or well defined margins in plain radiographs

X线平片表现为单发或多发溶骨性病灶，圆形或卵圆形，边界清晰或稍不清晰。

CT depicts expansive lytic bone destruction that may have a geographic pattern and soft tissue masses. Limited sclerosis is seen during the healing stage.

CT表现为膨胀性溶骨性骨质破坏，呈地图样，可见软组织肿块。愈合期时可见病灶周围硬化边。

On MRI most lesions have low-to-intermediate signal intensity on T1WI, intermediate-to-high on T2WI, and they enhance avidly with contrast.

MRI上，病灶T1WI通常表现为低至中等信号，T2WI呈中等或高信号，增强扫描明显强化。

Other findings include edema, paravertebral soft tissue components, and peridural compression.

其他表现包括水肿、椎旁软组织肿块、硬膜囊受压。

Vertebra plana may be seen on all these imaging modalities.

所有影像学检查均可见“扁平椎”改变。

鉴别诊断

Ewing sarcoma：More likely to have large soft tissue mass, permeative bone destruction and relative hypointense in T2

尤文氏肉瘤：通常软组织肿块较大，弥漫性骨质破坏，T2WI表现为相对低信号。

Lymphoma：Multiple levels, ivory vertebral body, hypointense-to-normal marrow

淋巴瘤：多椎体发病，椎体密度增高，呈“象牙椎”，MRI骨髓呈低或等信号。

Metastatic neuroblastoma：Multiple osteosclerotic lesions

转移性神经母细胞瘤：多发成骨性病灶。

Discitis-osteomyelitis：Hyperintense narrow disc with or without fluid signal

椎间盘炎-骨髓炎：椎间隙狭窄，呈高信号，可伴有或不伴有液性信号。

治疗要点

For monostotic disease, treatment includes observation, injection of corticosteroids, nonsteroidal anti-inflammatories, and surgical curettage.

对于单发病灶，主要治疗方法包括：随访观察、注射皮质激素、非甾体类抗炎药、手术刮除等。