Hillenbrand Thorsten, Menge Franka, Hohenberger Peter, Kasper Bernd
Sarcoma Unit, Interdisciplinary Tumor Center Mannheim, Mannheim University Medical Center, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.
Clin Sarcoma Res. 2015 May 23;5:14. doi: 10.1186/s13569-015-0028-9. eCollection 2015.
Angiosarcomas (AS) are rare vascular malignancies. They are subdivided into primary (PAS) and secondary angiosarcomas (SAS). The objective was to compare the characteristics of AS subtypes.
Eighteen PAS and ten SAS patients treated at our institution between 2004 and 2012 were included in this study.
Median age of PAS and SAS patients was 52.9 and 64.2 years, respectively (p = 0.1448). The percentage of women was 27.8% for PAS, but 80.0% for SAS (p = 0.0163). While PAS occurred throughout the body, the majority of SAS arose from the breast (p = 0.0012). All SAS were radiation-induced with a median latency of 7.7 years. The majority of patients with PAS and SAS underwent surgery as primary or recurrence treatment (p > 0.95). Local recurrence was developed by 27.8% of PAS and 50.0% of SAS (p = 0.4119). 61.1% of PAS metastasized, but only 40.0% of SAS (p = 0.4328). Median overall survival for PAS and SAS was 19 and 57 months, respectively (p = 0.2306).
Radical surgery remains the mainstay of both primary and recurrence treatment. SAS show a high local recurrence rate, while PAS tend towards developing early metastases. Overall, prognosis is poor for both groups.
血管肉瘤(AS)是一种罕见的血管恶性肿瘤。它们可细分为原发性血管肉瘤(PAS)和继发性血管肉瘤(SAS)。目的是比较AS亚型的特征。
本研究纳入了2004年至2012年在我们机构接受治疗的18例PAS患者和10例SAS患者。
PAS和SAS患者的中位年龄分别为52.9岁和64.2岁(p = 0.1448)。PAS患者中女性的比例为27.8%,而SAS患者中这一比例为80.0%(p = 0.0163)。PAS可发生于全身各处,而大多数SAS起源于乳腺(p = 0.0012)。所有SAS均由放疗引起,中位潜伏期为7.7年。大多数PAS和SAS患者接受手术作为主要或复发治疗(p > 0.95)。27.8%的PAS患者出现局部复发,50.0%的SAS患者出现局部复发(p = 0.4119)。61.1%的PAS发生转移,而只有40.0%的SAS发生转移(p = 0.4328)。PAS和SAS的中位总生存期分别为19个月和57个月(p = 0.2306)。
根治性手术仍然是原发性和复发性治疗的主要手段。SAS表现出较高的局部复发率,而PAS倾向于早期发生转移。总体而言,两组的预后都很差。