Katou Shadi, Di Pietro Martinelli Claudine, Silveira Carolina, Schmid Franziska, Becker Felix, Radunz Sonia, Juratli Mazen, Morgul Haluk, Banz Vanessa, Pascher Andreas, Andreou Andreas, Struecker Benjamin
Department for General, Visceral, and Transplant Surgery, University Hospital Muenster, 48149 Muenster, Germany.
Department of Visceral Surgery und Medicine, Inselspital, Bern University Hospital, University of Bern, 3010 Bern, Switzerland.
J Clin Med. 2022 May 25;11(11):2990. doi: 10.3390/jcm11112990.
Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the liver, and data on patient outcome after surgical treatment are scarce. The aim of this study was to evaluate postoperative morbidity and overall survival (OS) of patients who underwent hepatectomy for PHA. This is a bicentric retrospective analysis of all consecutive patients who underwent liver resection in curative intent for PHA between 2012 and 2019 at the University Hospital of Muenster and the University Hospital of Bern. Nine patients (five female, four male) were included from both centers. Median age was 72 years (44-82). Most lesions (77.8%) were larger than 5 cm, and mean size of the biggest lesion was 9.4 ± 4.5 cm. Major hepatectomy was performed in four (44.4%), and radical resection (R0) was achieved in six (66.7%) patients. Postoperative complication rate was 88.8%, including 44.4% higher than 3a in the Clavien-Dindo classification. OS survival rates at 1, 2, and 3 years were 44.4%, 22.2%, and 12.5%, respectively, and median OS was 5 months. OS was significantly better after radical resection (R0: 15 months vs. R1: 0 months, = 0.04), whereas presentation with tumor rupture at diagnosis was associated with the worst OS (0 months vs. 15 months, = 0.005). Disease recurrence occurred in three patients (33.3%) between three and seven months after surgery. Radical resection remains the only potentially curative treatment option for PHA. However, postoperative morbidity is high, and the overall prognosis remains poor. Multimodal therapy options and management strategies are urgently needed and could improve the prognosis of patients suffering from PHA in the future.
原发性肝血管肉瘤（PHA）是一种罕见的肝脏恶性肿瘤，关于手术治疗后患者预后的数据很少。本研究的目的是评估接受肝切除术治疗PHA患者的术后发病率和总生存期（OS）。这是一项双中心回顾性分析，纳入了2012年至2019年间在明斯特大学医院和伯尔尼大学医院接受根治性肝切除术治疗PHA的所有连续患者。两个中心共纳入9例患者（5例女性，4例男性）。中位年龄为72岁（44 - 82岁）。大多数病变（77.8%）大于5 cm，最大病变的平均大小为9.4±4.5 cm。4例（44.4%）患者接受了扩大肝切除术，6例（66.7%）患者实现了根治性切除（R0）。术后并发症发生率为88.8%，其中Clavien - Dindo分类中高于3a级的并发症发生率为44.4%。1年、2年和3年的OS生存率分别为44.4%、22.2%和12.5%，中位OS为5个月。根治性切除（R0：15个月 vs. R1：0个月，P = 0.04）后的OS明显更好，而诊断时出现肿瘤破裂与最差的OS相关（0个月 vs. 15个月，P = 0.005）。3例患者（33.3%）在术后3至7个月出现疾病复发。根治性切除仍然是PHA唯一潜在的治愈性治疗选择。然而，术后发病率高，总体预后仍然很差。迫切需要多模式治疗方案和管理策略，未来可能改善PHA患者的预后。
