脂肪肉瘤

J. Crompton, Arun S. Singh, S. Dry, F. Eilber
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摘要

脂肪肉瘤是一种间质恶性肿瘤,主要发生在腹膜后、躯干和四肢。脂肪肉瘤的组织学亚型包括高分化/去分化、粘液样/圆细胞和多形性脂肪肉瘤。虽然脂肪肉瘤很少见,几乎可以发生在任何解剖位置,但它是腹膜后最常见的软组织肉瘤,手术仍然是唯一可能治愈的治疗方法。本文就脂肪肉瘤的发病机制、分类、临床诊断评价、治疗和监测进行综述。治疗的基石是手术,其主要治疗目标是提高生存率,限制局部复发,最大限度地提高功能,并尽量减少发病率。围手术期放射治疗可能适用于大的高级别脂肪肉瘤患者。全身治疗——无论是传统的化疗还是分子靶向治疗——可能根据患者的组织学亚型、部位和疾病程度而受益。然而,对于大多数患者来说,全身治疗是无法治愈的。在脂肪肉瘤的管理争议的问题,如手术边缘和切除的程度,也进行了讨论。关键词:非典型脂肪性脂肪肉瘤、脂肪肉瘤、黏液性脂肪肉瘤、多形性脂肪肉瘤、腹膜后肉瘤、软组织肉瘤、高分化/去分化脂肪肉瘤
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Liposarcoma
Liposarcoma is a mesenchymal malignancy that predominantly arises in the retroperitoneum, trunk, and extremities. Histologic subtypes of liposarcoma include well-differentiated/dedifferentiated, myxoid/round cell, and pleomorphic liposarcoma. Although liposarcoma is rare and can occur in almost any anatomic location, it is the most common soft tissue sarcoma in the retroperitoneum, and surgery remains the only potentially curative therapy. This review discusses liposarcoma pathogenesis, classification, clinical-diagnostic evaluation, treatment, and surveillance. The cornerstone of treatment is surgery, with the main therapeutic goals of improving survival, limiting local recurrence, maximizing function, and minimizing morbidity. Perioperative radiation therapy may be indicated in patients with large high-grade liposarcomas. Systemic treatment—whether traditional chemotherapy or molecularly targeted therapy—may benefit patients depending on their histologic subtype, location, and extent of disease. For the majority of patients, however, systemic treatment is not curative. Controversial issues in the management of liposarcoma, such as surgical margins and extent of resection, are also discussed.    This review contains 2 figures, 5 tables and 39 references Key words: atypical lipomatous liposarcoma, liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, retroperitoneal sarcoma, soft tissue sarcoma, well-differentiated/dedifferentiated liposarcoma
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