Abraham John A, Hornicek Francis J, Kaufman Adam M, Harmon David C, Springfield Dempsey S, Raskin Kevin A, Mankin Henry J, Kirsch David G, Rosenberg Andrew E, Nielsen G Petur, Desphpande Vikram, Suit Herman D, DeLaney Thomas F, Yoon Sam S
Orthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114, USA.
Ann Surg Oncol. 2007 Jun;14(6):1953-67. doi: 10.1245/s10434-006-9335-y. Epub 2007 Mar 14.
Angiosarcomas are an uncommon type of malignancy that are generally thought to behave usually in a locally aggressive fashion; they often metastasize to distant sites.
Patients with a diagnosis of angiosarcoma treated at our institution between 1980 and 2006 were analyzed for patient demographics, tumor characteristics, multimodality treatment, and outcomes.
A total of 82 patients were divided into those with primary and advanced disease. Overall, the median age was 65 (range, 22-91) years, and 44% of patients were women. Median size of tumors was 3.8 cm, and 76% of tumors were intermediate or high grade. Tumors were located throughout the body: 32 cutaneous, 22 deep soft tissues or organs, 10 radiation or lymphedema field, 8 bone, and 7 nonirradiated breast. Of 46 patients with primary disease, all patients underwent surgical resection, 67% received radiotherapy, and 27% received chemotherapy. Five-year disease-specific survival was 60%, and negative prognostic factors included intermediate or high grade, and tumors arising in a radiated or lymphedema field. Of 36 patients with advanced disease, 36% underwent a palliative operation, 78% received radiation, and 58% received chemotherapy. Median survival was just 7.3 months, and cutaneous tumors predicted a better prognosis compared with other sites.
Primary angiosarcomas treated with aggressive surgical resection and the addition of radiation for close margins or worrisome pathologic features can result in long-term survival in most patients. The role of adjuvant chemotherapy is unclear. Patients with advanced disease have a poor prognosis, but there can be dramatic responses to chemotherapy in a minority of patients.
血管肉瘤是一种罕见的恶性肿瘤，通常被认为具有局部侵袭性；它们常转移至远处部位。
对1980年至2006年在我们机构接受治疗的血管肉瘤患者进行分析，包括患者人口统计学特征、肿瘤特征、多模式治疗及治疗结果。
共82例患者被分为原发性疾病组和晚期疾病组。总体而言，中位年龄为65岁（范围22 - 91岁），44%为女性。肿瘤中位大小为3.8厘米，76%的肿瘤为中级或高级别。肿瘤分布于全身各处：32例位于皮肤，22例位于深部软组织或器官，10例位于放疗或淋巴水肿区域，8例位于骨骼，7例位于未接受放疗的乳腺。46例原发性疾病患者中，所有患者均接受了手术切除，67%接受了放疗，27%接受了化疗。5年疾病特异性生存率为60%，不良预后因素包括中级或高级别以及放疗或淋巴水肿区域发生的肿瘤。36例晚期疾病患者中，36%接受了姑息性手术，78%接受了放疗，58%接受了化疗。中位生存期仅7.3个月，与其他部位相比，皮肤肿瘤预后较好。
对于原发性血管肉瘤，积极的手术切除并对切缘阳性或病理特征令人担忧的患者加用放疗，可使大多数患者获得长期生存。辅助化疗的作用尚不清楚。晚期疾病患者预后较差，但少数患者对化疗可能有显著反应。
