Levy Angela D, Manning Maria A, Al-Refaie Waddah B, Miettinen Markku M
From the Departments of Radiology (A.D.L., M.A.M.) and Surgery (W.B.A.), Medstar Georgetown University Hospital, 3800 Reservoir Rd NW, Washington, DC 20007; the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.); and the Center for Cancer Research, National Cancer Institute, Bethesda, Md (M.M.M.).
Radiographics. 2017 Mar-Apr;37(2):462-483. doi: 10.1148/rg.2017160157.
Soft-tissue sarcomas are a diverse group of rare mesenchymal malignancies that can arise at any location in the body and affect all age groups. These sarcomas are most common in the extremities, trunk wall, retroperitoneum, and head and neck. In the adult population, soft-tissue sarcomas arising in the abdomen and pelvis are often large masses at the time of diagnosis because they are usually clinically silent or cause vague or mild symptoms until they invade or compress vital organs. In contrast, soft-tissue sarcomas arising from the abdominal wall come to clinical attention earlier in the course of disease because they cause a palpable mass, abdominal wall deformity, or pain that is more clinically apparent. The imaging features of abdominal and pelvic sarcomas and abdominal wall sarcomas can be nonspecific and overlap with more common pathologic conditions, making diagnosis difficult or, in some cases, delaying diagnosis. Liposarcoma (well-differentiated and dedifferentiated liposarcomas), leiomyosarcoma, and gastrointestinal stromal tumor (GIST) are the most common intra-abdominal primary sarcomas. Any soft-tissue sarcoma can arise in the abdominal wall. Knowledge of the classification and pathologic features of soft-tissue sarcomas, the anatomic locations where they occur, and their cross-sectional imaging features helps the radiologist establish the diagnosis or differential diagnosis so that patients with soft-tissue sarcomas can receive optimal treatment and management. In part 1 of this article, the most common soft-tissue sarcomas (liposarcoma, leiomyosarcoma, and GIST) are reviewed, with a discussion on anatomic locations, classification, clinical considerations, and differential diagnosis. Part 2 will focus on the remainder of the soft-tissue sarcomas occurring in the abdomen and pelvis.
软组织肉瘤是一组多样的罕见间叶性恶性肿瘤,可发生于身体的任何部位,影响所有年龄组。这些肉瘤最常见于四肢、躯干壁、腹膜后以及头颈部。在成人中,发生于腹部和骨盆的软组织肉瘤在诊断时通常是大肿块,因为它们通常在临床上无症状,或在侵犯或压迫重要器官之前仅引起模糊或轻微的症状。相比之下,腹壁来源的软组织肉瘤在疾病过程中更早引起临床关注,因为它们会导致可触及的肿块、腹壁畸形或更明显的临床疼痛。腹部和盆腔肉瘤以及腹壁肉瘤的影像学特征可能不具有特异性,且与更常见的病理状况重叠,这使得诊断困难,在某些情况下还会延迟诊断。脂肪肉瘤(高分化和去分化脂肪肉瘤)、平滑肌肉瘤和胃肠道间质瘤(GIST)是最常见的腹内原发性肉瘤。任何软组织肉瘤都可能发生在腹壁。了解软组织肉瘤的分类和病理特征、其发生的解剖位置以及横断面影像学特征,有助于放射科医生进行诊断或鉴别诊断,以便软组织肉瘤患者能够接受最佳的治疗和管理。在本文的第1部分中,将对最常见的软组织肉瘤(脂肪肉瘤、平滑肌肉瘤和GIST)进行综述,并讨论其解剖位置、分类、临床考虑因素和鉴别诊断。第2部分将聚焦于发生在腹部和骨盆的其余软组织肉瘤。