Lee Biing Luen, Chen Cheng-Feng, Chen Paul Chih-Hsueh, Lee Hui-Chen, Liao Wen-Chieh, Perng Chern-Kang, Ma Hsu, Lin Chih-Hsun
From the *Department of Surgery, Veterans General Hospital Yuanshan & Su-Ao Branch; †Division of Plastic Surgery, Department of Surgery, Taipei Veterans General Hospital; ‡Department of Surgery, School of Medicine, National Yang-Ming University, Taipei; §Department of Surgery, Koo Foundation Sun Yat-Sen Cancer Center; and ∥Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taiwan, Republic of China.
Ann Plast Surg. 2017 Mar;78(3 Suppl 2):S41-S46. doi: 10.1097/SAP.0000000000001004.
Primary cutaneous and soft tissue angiosarcoma is a rare but highly aggressive malignancy. To date, surgical resection is the mainstay of treatment, but poor prognosis is expected. To investigate whether there are factors associated with poor prognosis after surgical resection and to develop a treatment guideline for current therapy, we retrospectively collected data on 28 patients who underwent surgery as initial treatment and reviewed patient demographics, tumor characteristics, disease courses, and prognoses from September 1996 to May 2013. Of these 28 patients, 17 (60.7%) were men and the mean age at first diagnosis was 66.57 ± 18.57 years. Anatomically, 17 (60.7%) tumors were in the scalp and 11 (39.3%) were in other sites of the body. Of the 28 patients, 23 (82.1%) had achieved negative surgical margins, 24 (85.7%) received adjuvant radiation therapy, and 17 (60.7%) received adjuvant chemotherapy. Twenty-one patients (75%) died during a mean follow-up time of 35.86 ± 28.91 months, and all deaths were caused by angiosarcoma. The 5-year overall survival rate was 17.86%. Sixteen (57.1%) patients had locoregional tumor recurrence, and 20 (71.4%) had distant metastases, with a median of 9.17 (range, 1.9-98.07) months to recurrence or metastasis. Possible predictors of poor prognosis (P < 0.05) in terms of disease-free survival after surgical resection were male sex, cardiovascular disease, smoking, and scalp angiosarcomas, those in terms of overall survival were older than 70 years, male sex, cardiovascular disease, smoking, scalp angiosarcomas, distant metastases, and not receiving adjuvant chemotherapy. In conclusion, although multimodal treatments are used, the overall prognosis after surgical resection is still poor, especially for patients with the above predictive factors. An early diagnosis and complete resection of the primary tumor with or without adjuvant radiotherapy and chemotherapy are suggested for a potential better outcome. For those who have a diffuse lesion pattern with the involvement of vital structures, recurrence, or metastasis, palliative resection could be an alternative treatment choice.
原发性皮肤和软组织血管肉瘤是一种罕见但侵袭性很强的恶性肿瘤。迄今为止，手术切除是主要的治疗方法，但预后较差。为了研究手术切除后是否存在与预后不良相关的因素，并制定当前治疗的指南，我们回顾性收集了1996年9月至2013年5月期间28例接受手术作为初始治疗的患者的数据，并对患者的人口统计学、肿瘤特征、病程和预后进行了回顾。这28例患者中，17例（60.7%）为男性，首次诊断时的平均年龄为66.57±18.57岁。从解剖学上看，17例（60.7%）肿瘤位于头皮，11例（39.3%）位于身体其他部位。28例患者中，23例（82.1%）手术切缘阴性，24例（85.7%）接受了辅助放疗，17例（60.7%）接受了辅助化疗。21例患者（75%）在平均35.86±28.91个月的随访期内死亡，所有死亡均由血管肉瘤引起。5年总生存率为17.86%。16例（57.1%）患者出现局部肿瘤复发，20例（71.4%）出现远处转移，复发或转移的中位时间为9.17（范围1.9 - 98.07）个月。手术切除后无病生存方面预后不良的可能预测因素（P < 0.05）为男性、心血管疾病、吸烟和头皮血管肉瘤，总生存方面的预测因素为年龄大于70岁、男性、心血管疾病、吸烟、头皮血管肉瘤、远处转移以及未接受辅助化疗。总之，尽管采用了多模式治疗，但手术切除后的总体预后仍然很差，尤其是对于具有上述预测因素的患者。建议早期诊断并完整切除原发性肿瘤，联合或不联合辅助放疗和化疗，以获得更好的潜在疗效。对于那些具有弥漫性病变模式且累及重要结构、复发或转移的患者，姑息性切除可能是一种替代治疗选择。
