Mark R J, Tran L M, Sercarz J, Fu Y S, Calcaterra T C, Juillard G F
Radiation Medical Group, San Diego, California.
Arch Otolaryngol Head Neck Surg. 1993 Sep;119(9):973-8. doi: 10.1001/archotol.1993.01880210061009.
Between 1955 and 1990, 28 patients with angiosarcoma of the head and neck were seen at UCLA Medical Center, Los Angeles, Calif. Half the lesions arose on the scalp; the remainder occurred in the soft tissues of the face and neck. Nine patients presented with multifocal disease. Follow-up ranged from 3 to 159 months, with a median of 32 months. The overall prognosis was poor, with a 5-year disease-free survival of 26% (7/27 patients). Of 21 patients having recurrences after primary treatment, 90% (19/21 patients) had a component of local failure. Distant metastasis had developed in nine patients at last follow-up. Of patients treated initially with surgery alone, 8% (1/12 patients) remain disease free vs 67% (4/6 patients) who received postoperative radiation therapy, with or without chemotherapy. Only one (14%) of seven patients treated primarily with radiation therapy with or without chemotherapy was rendered disease free. Angiosarcoma usually presents with high-grade histologic features and frequently with multifocal disease. There is propensity for both local recurrence and distant metastasis. Our results and a review of the literature suggest that combined modality therapy offers the best chance for long-term control in patients with angiosarcoma of the head and neck.
1955年至1990年间，加利福尼亚州洛杉矶市加州大学洛杉矶分校医疗中心收治了28例头颈部血管肉瘤患者。半数病损发生于头皮，其余发生于面部和颈部软组织。9例患者表现为多灶性病变。随访时间为3至159个月，中位随访时间为32个月。总体预后较差，5年无病生存率为26%（27例患者中的7例）。在21例初始治疗后复发的患者中，90%（21例患者中的19例）有局部失败成分。在最后一次随访时，9例患者出现远处转移。仅接受手术治疗的患者中，8%（12例患者中的1例）无病生存，而接受术后放疗（无论是否联合化疗）的患者中，这一比例为67%（6例患者中的4例）。在7例主要接受放疗（无论是否联合化疗）的患者中，只有1例（14%）实现了无病生存。血管肉瘤通常表现为高级别组织学特征，且常为多灶性病变。有局部复发和远处转移的倾向。我们的结果以及文献回顾表明，综合治疗模式为头颈部血管肉瘤患者提供了最佳的长期控制机会。
