Otero S, Moskovic E C, Strauss D C, Benson C, Miah A B, Thway K, Messiou C
Department of Radiology and Soft Tissue Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London SW3 6JJ, UK.
Department of Radiology and Soft Tissue Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London SW3 6JJ, UK.
Clin Radiol. 2015 Sep;70(9):1038-45. doi: 10.1016/j.crad.2015.04.015. Epub 2015 Jul 7.
Desmoid-type fibromatosis is a rare, locally infiltrative, mesenchymal neoplasm that is associated with high rates of local recurrence but lacks the potential to metastasise. The disease affects younger individuals, with a peak age of 30 years, and is the most common cause of an anterior abdominal wall mass in young women of childbearing age. It may, however, involve nearly every body part, including the extremities, head and neck, trunk, and abdominal cavity; as such, desmoid-type fibromatosis may present to a range of general and subspecialty radiologists. These rare tumours have a widely variable clinical presentation and unpredictable natural history, hence input from a soft-tissue tumour centre is recommended, although much of the imaging may be performed at the patient's local hospital. The consensus for treatment has changed over the past decade, with most centres moving away from primary radical surgery towards a front-line 'watch-and-wait' policy. Therefore, imaging has an increasingly important role to play in both the diagnosis and follow-up of these patients. This review will discuss the typical imaging characteristics of these lesions and suggest diagnostic and follow-up magnetic resonance imaging protocols, with details of suitable sequences and scanning intervals.
韧带样型纤维瘤病是一种罕见的、局部浸润性的间叶肿瘤,局部复发率高,但无转移潜能。该疾病好发于年轻人,发病高峰年龄为30岁,是育龄期年轻女性前腹壁肿块最常见的原因。然而,它几乎可累及身体的各个部位,包括四肢、头颈部、躯干和腹腔;因此,韧带样型纤维瘤病可能会被不同的普通放射科医生和专科放射科医生诊断。这些罕见肿瘤的临床表现差异很大,自然病程不可预测,因此建议咨询软组织肿瘤中心,尽管大部分影像学检查可能在患者当地医院进行。在过去十年中,治疗共识发生了变化,大多数中心已从原发性根治性手术转向一线“观察等待”策略。因此,影像学在这些患者的诊断和随访中发挥着越来越重要的作用。本综述将讨论这些病变的典型影像学特征,并提出诊断和随访磁共振成像方案,包括合适的序列和扫描间隔的详细信息。