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从 AFIP 档案中:肌肉骨骼纤维瘤病:放射学-病理学相关性。

From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.

机构信息

Department of Radiologic Pathology, Armed Forces Institute of Pathology, Building 54, Room M-133A, Washington, DC 20306, USA.

出版信息

Radiographics. 2009 Nov;29(7):2143-73. doi: 10.1148/rg.297095138.

Abstract

Musculoskeletal fibromatoses represent a wide spectrum of fibroblastic and myofibroblastic neoplasms with similar pathologic appearances and variable clinical behavior. These lesions can be categorized by location (superficial or deep) or by the age group predominantly affected. Superficial fibromatoses in adults (palmar and plantar) and children (calcifying aponeurotic fibroma, lipofibromatosis, and inclusion body fibromatosis) are often small slow-growing lesions; their diagnosis is suggested by location. Deep fibromatoses in adults (desmoid type and abdominal wall) and children (fibromatosis colli and myofibroma and myofibromatosis) are frequently large and more rapidly enlarging; location of these lesions may be nonspecific. Radiographic findings typically are nonspecific. Cross-sectional imaging (ultrasonography, computed tomography, or magnetic resonance [MR] imaging) reveals lesion location, extent, and involvement of adjacent structures for staging and evaluation of local recurrence. MR imaging findings of predominantly low to intermediate signal intensity, nonenhancing bands of low signal intensity on long repetition time MR images that represent collagenized regions, and extension along fascial planes ("fascial tail" sign) add specificity for diagnosis. Additional features that aid in diagnostic specificity include an abdominal wall location related to pregnancy (abdominal wall fibromatosis), a lower neck location in a young child (fibromatosis colli), an adipose component (lipofibromatosis), or multiple lesions in young children (myofibromatosis). Treatment may be conservative or surgical resection, depending on the specific diagnosis. Local recurrence is common after surgical resection owing to the infiltrative growth of these lesions. Recognition that the appearances of the various types of musculoskeletal fibromatoses reflect their pathologic characteristics improves radiologic assessment and helps optimize patient management.

摘要

肌肉骨骼纤维瘤病代表了一系列具有相似病理表现和不同临床行为的纤维母细胞和肌纤维母细胞肿瘤。这些病变可根据位置(表浅或深部)或主要受影响的年龄组进行分类。成人(手掌和足底)和儿童(钙化性腱膜纤维瘤、脂肪纤维瘤病和包涵体纤维瘤病)的表浅纤维瘤病通常是生长缓慢的小病变;其诊断取决于病变的位置。成人(硬纤维瘤型和腹壁)和儿童(颈纤维瘤病和肌纤维瘤和肌纤维瘤病)的深部纤维瘤病常较大且生长迅速;这些病变的位置可能不具有特异性。影像学表现通常是非特异性的。横断面成像(超声、计算机断层扫描或磁共振成像)可显示病变的位置、范围以及相邻结构的受累情况,用于分期和评估局部复发。磁共振成像表现主要为低到中等信号强度,长重复时间磁共振图像上的低信号强度非增强带代表胶原化区域,以及沿着筋膜平面延伸(“筋膜尾”征)增加了诊断的特异性。有助于提高诊断特异性的其他特征包括与妊娠相关的腹壁位置(腹壁纤维瘤病)、幼儿的下颈部位置(颈纤维瘤病)、脂肪成分(脂肪纤维瘤病)或幼儿的多个病变(肌纤维瘤病)。治疗方法取决于具体的诊断,可以是保守治疗或手术切除。由于这些病变呈浸润性生长,手术后局部复发很常见。认识到各种类型的肌肉骨骼纤维瘤病的表现反映了其病理特征,可以提高影像学评估并有助于优化患者管理。

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