Tripke Verena, Heinrich Stefan, Huber Tobias, Mittler Jens, Hoppe-Lotichius Maria, Straub Beate K, Lang Hauke
Department of General, Visceral and Transplantation Surgery, University Hospital of Mainz, Langenbeckstrasse 1, 55131, Mainz, Germany.
Institute of Pathology, University Hospital of Mainz, Mainz, Germany.
BMC Surg. 2019 Jan 10;19(1):5. doi: 10.1186/s12893-018-0465-5.
Primary hepatic angiosarcoma (PHA) is a rare tumor entity. Radical surgical resection is currently considered the best treatment choice. The aim of this analysis is to report our experience with surgery for PHA.
All resections of PHA from 01/2002 until 06/2017 were identified from our prospective institutional database. All cases were re-confirmed by a second pathologist. We analyzed completeness of resection, overall (OS) and disease-free survival (DFS).
Nine patients with PHA underwent hepatic resection. Median follow-up after surgery was 15.5 months (range: 3-144). At last follow-up 4/9 patients were alive, three of them without recurrence 15, 21 and 144 months after surgery. Five patients developed PHA recurrence. Four of these died 3 to 17 months after surgery. One patient with PHA recurrence is alive 15 months after surgery. Another patient without PHA recurrence died 59 months after surgery from pancreatic cancer. Median OS and DFS after resection was 18 months (range: 3-144 months) and 10 months (range: 2-144 months), respectively. After R-0 resection (n = 8), the median OS and DFS was 59 and 11 months.
Resection of PHA is the only approach to achieve complete tumor removal and offers a chance for long-term survival and should be evaluated in cases of PHA.
原发性肝血管肉瘤（PHA）是一种罕见的肿瘤实体。根治性手术切除目前被认为是最佳治疗选择。本分析的目的是报告我们治疗PHA的手术经验。
从我们的前瞻性机构数据库中识别出2002年1月至2017年6月期间所有PHA切除术病例。所有病例均由另一位病理学家再次确认。我们分析了切除的完整性、总生存期（OS）和无病生存期（DFS）。
9例PHA患者接受了肝切除术。术后中位随访时间为15.5个月（范围：3 - 144个月）。在最后一次随访时，9例患者中有4例存活，其中3例在术后15、21和144个月无复发。5例患者发生PHA复发。其中4例在术后3至17个月死亡。1例PHA复发患者术后15个月存活。另1例无PHA复发的患者术后59个月死于胰腺癌。切除术后的中位OS和DFS分别为18个月（范围：3 - 144个月）和10个月（范围：2 - 144个月）。R-0切除（n = 8）后，中位OS和DFS分别为59个月和11个月。
PHA切除术是实现肿瘤完全切除的唯一方法，为长期生存提供了机会，对于PHA病例应进行评估。
