Primary Breast Angiosarcoma-＂Fifteen Years of Clinical Experience at Taipei Veterans General Hospital and Koo Foundation Sun Yat-Sen Cancer Center＂

原發性乳房惡性血管肉瘤－台北榮民總醫院及和信治癌中心醫院之15年治療經驗

李秉侖(Biing-Luen Lee) ； 陳呈峰(Cheng-Feng Chen) ； 馬旭(Hsu Ma) ； 廖文傑(Wen-Chieh Liao)

《臺灣整形外科醫學會雜誌》 24卷1期 (2015/03) Pp. 20-28

摘要

背景：乳房血管肉瘤是一種極為少見、預後差的惡性腫瘤。依據是否接受先前的乳癌放射線治療又分為原發性和次發性乳房血管肉瘤。相較於次發性乳房惡性血管肉瘤，較少文獻討論到原發性乳房惡性血管肉瘤之臨床表現及預後，其中更缺乏亞洲人種的相關數據。目的及目標：藉由兩家醫學中心十五年的經驗分析病人臨床、病理學的表現及預後。材料及方法：藉由回溯性的病例分析統計自1996至2011年兩家醫學中心15年期間，共7位病理診斷為乳房惡性血管肉瘤之女性病患（兩位在台北榮總，五位在和信醫院）。分析的內容包含病人基本資料、臨床及病理學之表現、治療方式及預後。結果：此7位病患先前皆未有罹患乳癌及接受乳房放射線治療之病史。年齡中位數為44歲（31-51歲），腫瘤直徑中位數5.4公分（2-15公分）。病人一開始的臨床表現皆為乳房腫塊或腫脹，其中3位病人伴隨紫紅色的皮膚斑疹。6位病患在此二間醫學中心接受全乳切除（另一位未接受手術且未再回診之病人被排除），其中3未發生局部性或區域性復發；4位有遠端轉移，轉移部位為肺部、肝臟及骨頭。5位接受放射治療及化療、1位只接受放射治療。在中位數為26.5個月（10-127個月）的追蹤期間內共三位死亡（50%），死亡皆直接歸因於腫瘤。結論：原發性乳房惡性血管肉瘤好發於健康之中年女性，起始症狀大多是乳房腫塊伴隨紫紅色之皮膚病灶，易與外傷性血腫混淆造成診斷的困難及增加延遲治療的機會。核磁共振為推薦使用的診斷方式，而針對臨床上高度懷疑的病人，盡快做病理切片檢查是唯一的確診方式。此種腫瘤少見且惡性度高，即使完全切除其預後仍然很差。最佳的治療方式為全乳切除或廣泛切除至病理上完全陰性之邊緣狀態。

並列摘要

Background: Angiosarcoma of the breast is a rare malignancy with a poor prognosis. It presents as primary or secondary, the difference being a previous radiation-treated breast malignancy in the latter case. In contrast to secondary breast angiosarcoma, primary breast angiosarcoma has not been fully investigated, especially in Asians. Aim and Objectives: To evaluate the clinicopathologic characteristics and clinical outcomes of patients with primary breast angiosarcoma. Materials and Methods: We analyzed the clinicopathologic factors of patients with primary breast angiosarcoma pathologically diagnosed between 1996 and 2011 at Taipei Veterans General Hospital (VGHTPE) and Koo Foundation Sun Yat-Sen Cancer Center (KFSYSCC). We reviewed the related demographic and survival data, treatment modalities, and tumor focality, size, histologic grade, recurrence, and metastasis. Results: Seven women with primary angiosarcoma of the breast were included in our study, 2 at VGHTPE, and 5 at KFSYSCC; all cases were defined as angiosarcoma of the breast without previous radiation treatment for breast carcinoma. The median age of the patients was 44 years (range: 31–51 years), and the median tumor size was 5.4 cm (range: 2.0–15.0 cm). The initial presentation of the disease is breast mass/swelling, and 3 of the 7 patients with violaceous skin color lesions coexist. Six patients received simple mastectomy at least once at our institutes. (1 patient did not receive a mastectomy and was lost during follow up.) Amount the 6 patients, 3 presented with local/ regional recurrence, 4 with distant metastasis (lung, liver and bone), and all patients received radiation therapy and chemotherapy except one who received radiation therapy alone. Three patients (50%) died within a median follow-up time of 26.5 months (range: 10-a127 months), and all of these deaths were directly attributed to angiosarcoma. Conclusion: Primary breast angiosarcoma occurs in middle-aged, healthy women and initially presents as a mass and skin rash lesion that is easily misdiagnosed as a traumatic hematoma. Magnetic resonance imaging is the diagnostic test of choice, and biopsies of all suspicious lesions are warranted. The role of neo-adjuvant and adjuvant therapies remained limited value. Mastectomy or excision to negative margins appears to be the most appropriate and beneficial treatment.

並列關鍵字

angiosarcoma ； breast ； primary breast angiosarcoma ； secondary breast angiosarcoma