Martinez Salutario J, Moreno Courtney Coursey, Vinson Emily N, Dodd Leslie G, Brigman Brian E
Department of Radiology, Duke University Medical Center, Box 3808 Erwin Road, Durham, NC, 27710, USA.
Department of Radiology and Imaging Sciences, Emory University School of Medicine, 1364 Clifton Road, NE, Atlanta, GA, 30322, USA.
Skeletal Radiol. 2016 May;45(5):661-70. doi: 10.1007/s00256-016-2344-4. Epub 2016 Feb 27.
To describe novel MR imaging features, and clinical characteristics of soft tissue angiomatoid fibrous histiocytoma (AFH) at presentation, local recurrence, and metastases.
We described the MRI findings of six cases of histologically proven AFH. Pathologic findings, clinical presentation, and outcome were reviewed.
Lesions were primarily cystic. At initial presentation, tumors were surrounded by low signal intensity fibrous pseudocapsule. High signal intensity consistent with the lymphoplasmacytic infiltrate was seen in T2-weighted and post-contrast images as a rim over the hypointense pseudocapsule (double rim sign). High signal intensity infiltrating tumoral cords extended into adjacent tissues, through pseudocapsular defects on T2-weighted and post-contrast images. The cystic component and tumor cell nodularity were demonstrated at post-contrast images. Clinically, lesions were often thought to be benign, underwent marginal resection, developed local recurrence, and one developed second recurrence consisting of metastases. Recurrent tumors appeared as multiple masses, misinterpreted as post-surgical changes. An intramuscular recurrence demonstrated double rim and infiltrating margin.
A predominantly well-circumscribed, primarily cystic mass with double-rim and marginal infiltration on MRI suggests the possibility of AFH, in particular in child or young adult. Inclusion of these novel observations in AFH differential diagnosis may have a significant impact on treatment and prevention of recurrence.
描述软组织血管样纤维组织细胞瘤（AFH）在初诊、局部复发及转移时的新型磁共振成像（MR）特征及临床特点。
我们描述了6例经组织学证实的AFH的MRI表现。回顾了病理结果、临床表现及预后。
病变主要为囊性。初诊时，肿瘤被低信号强度的纤维假包膜包绕。在T2加权像及增强后图像上，与淋巴浆细胞浸润一致的高信号强度表现为低信号假包膜上的一层边缘（双边缘征）。在T2加权像及增强后图像上，高信号强度的浸润性肿瘤条索通过假包膜缺损延伸至相邻组织。增强后图像显示了囊性成分及肿瘤细胞结节。临床上，病变常被认为是良性的，行边缘切除，发生局部复发，其中1例出现包括转移的二次复发。复发肿瘤表现为多个肿块，被误诊为术后改变。1例肌肉内复发表现为双边缘及浸润性边缘。
MRI上主要表现为边界清晰、以囊性为主、具有双边缘及边缘浸润的肿块提示AFH的可能性，尤其是在儿童或年轻成人中。将这些新发现纳入AFH的鉴别诊断可能对治疗及预防复发有重大影响。
