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朗格汉斯细胞组织细胞增多症:34例中观察到的不寻常骨骼表现

Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases.

作者信息

Hindman B W, Thomas R D, Young L W, Yu L

机构信息

Loma Linda University Medical Center, CA 92354, USA.

出版信息

Skeletal Radiol. 1998 Apr;27(4):177-81. doi: 10.1007/s002560050361.

DOI:10.1007/s002560050361
PMID:9592898
Abstract

OBJECTIVE

Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material.

DESIGN AND PATIENTS

Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion.

RESULTS

Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial 'button' sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet.

CONCLUSIONS

Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis.

摘要

目的

朗格汉斯细胞组织细胞增多症偶尔会出现不寻常的表现,可能会造成混淆。本研究的目的是确定我们病例资料中不寻常表现的发生频率。

设计与患者

回顾性分析34例经活检证实为朗格汉斯细胞组织细胞增多症(LCH)的儿童,平均年龄4.4岁(范围3个月至16岁),共有262处骨骼病变,以确定不寻常表现的发生频率,这些表现定义为骨骼病变的非典型部位或病变的非典型影像学表现。

结果

在本次回顾性分析中发现了24处不寻常病变。其中包括骨骺病变、穿骺病变、颅外“纽扣”死骨、椎弓后病变、椎体病变的硬脊膜延伸以及液-液平面。液-液平面的发现此前在放射学文献中尚未有描述。不寻常部位的受累包括锁骨以及手和脚的小骨。

结论

LCH的X线、计算机断层扫描和磁共振成像会产生各种不寻常的表现。认识到LCH的这些不同表现可能会避免将这些表现与其他病理过程混淆。当不寻常表现作为疾病的首发发现时,鉴别诊断应包括LCH。

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