Heitmann C, Ingianni G
Department of Plastic and Hand Surgery, Ferdinand Sauerbruch Klinikum Wuppertal, University of Düsseldorf, Germany.
Ann Plast Surg. 2000 Jan;44(1):72-5. doi: 10.1097/00000637-200044010-00012.
Lymphangiosarcoma (LAS) is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. Patients with LAS demonstrate a history of breast cancer treated by radical mastectomy in the majority of patients. In the 1960s the incidence of LAS in patients with a 5-year survival after radical mastectomy varied from 0.07 to 0.45%. Today, due to changes in the operative techniques of breast cancer, less chronic lymphedema is seen with only a scant number of LAS patients. The etiology of this enigmatic tumor is not yet completely understood. Histologically, LAS arises from vascular endotheliocytes, and all vascular sarcomas originating in the setting of a chronic lymphedema are categorized as LAS. There is no standard treatment of LAS. The treatment options include radical ablative surgery, radiation therapy, and chemotherapy. The prognosis of LAS is poor; long-term survival is the exception. Only early recognition and radical surgery offer a chance of cure.
淋巴管肉瘤（LAS）是一种发生于长期慢性淋巴水肿后的侵袭性恶性血管肿瘤。大多数LAS患者有乳腺癌根治术治疗史。在20世纪60年代，乳腺癌根治术后生存5年的患者中LAS发病率为0.07%至0.45%。如今，由于乳腺癌手术技术的改变，慢性淋巴水肿较少见，LAS患者数量也很少。这种神秘肿瘤的病因尚未完全明确。组织学上，LAS起源于血管内皮细胞，所有起源于慢性淋巴水肿背景下的血管肉瘤都归类为LAS。LAS尚无标准治疗方法。治疗选择包括根治性切除手术、放射治疗和化疗。LAS的预后很差；长期生存者罕见。只有早期识别并进行根治性手术才有治愈的机会。
