Sargar Kiran M, Sheybani Elizabeth F, Shenoy Archana, Aranake-Chrisinger John, Khanna Geetika
From the Mallinckrodt Institute of Radiology (K.M.S., E.F.S., G.K.) and Department of Pathology (A.S., J.A.C.), Washington University School of Medicine, 510 S Kingshighway Blvd, Campus Box 8131-MIR, St Louis, MO 63110.
Radiographics. 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191.
Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma). Imaging has a key role in the evaluation of lesion origin, extent, and involvement with adjacent structures, and in the treatment management and postresection surveillance of these tumors. The imaging findings of these tumors are often nonspecific. However, certain imaging features, such as low or intermediate signal intensity on T2-weighted magnetic resonance images and extension along fascial planes, support the diagnosis of a fibroblastic or myofibroblastic tumor. In addition, certain tumors have characteristic imaging findings (eg, multiple subcutaneous or intramuscular lesions in infantile myofibromatosis, plaquelike growth pattern of Gardner fibroma, presence of adipose tissue in lipofibromatosis) or characteristic clinical manifestations (eg, great toe malformations in fibrodysplasia ossificans fibroma, neonatal torticollis in fibromatosis colli) that suggest the correct diagnosis. Knowledge of the syndrome associations of some of these tumors-for example, the association between familial adenomatous polyposis syndrome and both Gardner fibroma and desmoid fibromatosis, and that between nevoid basal cell carcinoma syndrome and cardiac fibroma-further facilitate a diagnosis. The recognition of key imaging findings can help guide treatment management and help avoid unnecessary intervention in cases of benign lesions such as myositis ossificans and fibromatosis colli. In this article, we describe the various types of fibroblastic and myofibroblastic tumors in children and the characteristic clinical manifestations, imaging features, and growth patterns of these neoplasms-all of which aid in the appropriate radiologic assessment and management of these lesions. (©)RSNA, 2016.
小儿成纤维细胞性和肌成纤维细胞性肿瘤是一组相对常见的软组织增生性病变，其临床行为具有广泛的多样性。根据生物学行为，这些肿瘤可分为以下几类：良性（如骨化性肌炎、肌纤维瘤、颈部纤维瘤病）、中间型-局部侵袭性（如脂肪纤维瘤病、韧带样纤维瘤）、中间型-极少转移型（如炎性肌成纤维细胞肿瘤、婴儿纤维肉瘤、低级别肌成纤维细胞肉瘤）以及恶性（如纤维黏液样肉瘤、成人纤维肉瘤）。影像学在评估病变起源、范围及与相邻结构的关系，以及这些肿瘤的治疗管理和切除后监测方面起着关键作用。这些肿瘤的影像学表现通常不具有特异性。然而，某些影像学特征，如在T2加权磁共振图像上呈低或中等信号强度以及沿筋膜平面延伸，支持成纤维细胞性或肌成纤维细胞性肿瘤的诊断。此外，某些肿瘤具有特征性的影像学表现（如婴儿肌纤维瘤病中的多个皮下或肌内病变、加德纳纤维瘤的斑块样生长方式、脂肪纤维瘤病中脂肪组织的存在）或特征性的临床表现（如骨化性纤维发育异常中的大脚趾畸形、颈部纤维瘤病中的新生儿斜颈），这些有助于做出正确诊断。了解其中一些肿瘤与综合征的关联——例如，家族性腺瘤性息肉病综合征与加德纳纤维瘤和韧带样纤维瘤病之间的关联，以及痣样基底细胞癌综合征与心脏纤维瘤之间的关联——进一步有助于诊断。识别关键的影像学表现有助于指导治疗管理，并有助于避免对诸如骨化性肌炎和颈部纤维瘤病等良性病变进行不必要的干预。在本文中，我们描述了儿童各种类型的成纤维细胞性和肌成纤维细胞性肿瘤以及这些肿瘤的特征性临床表现、影像学特征和生长方式——所有这些都有助于对这些病变进行适当的放射学评估和管理。（©）RSNA，2016年
