Holden C A, Spittle M F, Jones E W
Cancer. 1987 Mar 1;59(5):1046-57. doi: 10.1002/1097-0142(19870301)59:5<1046::aid-cncr2820590533>3.0.co;2-6.
72 patients with angiosarcoma (AS) of the face and scalp have been analyzed with respect of various prognostic factors and the effects of different treatment regimes. This disease predominantly occurs in the elderly (age range, 56-92 years), and affects men rather more frequently than women (men: 44, women: 28). The clinical presentation varied, and included bruise-like lesions, dusky plaques, chronic edema or cellulitis, ulcerated nodules, pyoderma, and infected conditions. Due to lack of clinical awareness and problems with histologic assessment, delays in diagnosis were frequent. The majority of the tumors arose in the upper part of the face or scalp. Less commonly, the central part of the face was affected while only three tumors developed in the mandibular region. Overall the prognosis was poor; one half of the patients died within 15 months of presentation. Only 12% of the patients survived 5 years or more. Patients with lesions that were less than 10 cm in diameter responded better to treatment and statistically survived longer than those with larger lesions, emphasizing the crucial importance of early diagnosis. There is some indication that men, younger patients, patients with lesions on the central part of the face survived longer, but this was not of statistical significance. The histologic differentiation of the tumors at presentation also was of doubtful prognostic significance. Despite the overall poor prognosis, radical radiotherapy (mostly wide-field electron-beam therapy) in seven patients resulted in the apparent eradication of the local skin disease and prolonged survival of the patients. Pulmonary metastases developed 10 years later in two of those patients, however, but the face and scalp remained tumor-free.
对72例面部和头皮血管肉瘤（AS）患者进行了各种预后因素及不同治疗方案效果的分析。这种疾病主要发生在老年人中（年龄范围为56 - 92岁），男性患病比女性更常见（男性44例，女性28例）。临床表现多样，包括瘀斑样病变、暗斑、慢性水肿或蜂窝织炎、溃疡结节、脓皮病以及感染情况。由于临床认识不足和组织学评估存在问题，诊断延迟很常见。大多数肿瘤发生在面部或头皮的上部。较少见的是面部中央受累，而下颌区域仅出现3例肿瘤。总体预后较差；一半的患者在就诊后15个月内死亡。只有12%的患者存活5年或更长时间。直径小于10 cm的病变患者对治疗反应更好，统计学上比病变较大的患者存活时间更长，这强调了早期诊断的至关重要性。有迹象表明男性、年轻患者以及面部中央有病变的患者存活时间更长，但这并无统计学意义。肿瘤初诊时的组织学分化对预后的意义也存疑。尽管总体预后较差，但7例患者接受根治性放疗（大多为大野电子束治疗）后，局部皮肤疾病明显消除，患者存活期延长。然而，其中2例患者10年后出现肺转移，但面部和头皮仍无肿瘤。
