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Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation--the European Liver Transplant Registry experience.肝血管肉瘤:肝移植的绝对禁忌证——欧洲肝移植登记处的经验。
Transplantation. 2013 Mar 27;95(6):872-7. doi: 10.1097/TP.0b013e318281b902.
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The national cancer data base: past, present, and future.国家癌症数据库:过去、现在与未来。
Ann Surg Oncol. 2010 Jan;17(1):4-7. doi: 10.1245/s10434-009-0771-3. Epub 2009 Oct 22.
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Comparison of commission on cancer-approved and -nonapproved hospitals in the United States: implications for studies that use the National Cancer Data Base.美国癌症委员会批准与未批准医院的比较:对使用国家癌症数据库的研究的影响
J Clin Oncol. 2009 Sep 1;27(25):4177-81. doi: 10.1200/JCO.2008.21.7018. Epub 2009 Jul 27.
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Clinical features and treatment outcomes of advanced stage primary hepatic angiosarcoma.晚期原发性肝血管肉瘤的临床特征及治疗结果
Ann Oncol. 2009 Apr;20(4):780-7. doi: 10.1093/annonc/mdn702. Epub 2009 Jan 29.
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The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry.肝移植在肝上皮样血管内皮瘤治疗中的地位:欧洲肝移植登记处报告
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Management of primary liver sarcomas.原发性肝脏肉瘤的管理
Cancer. 2007 Apr 1;109(7):1391-6. doi: 10.1002/cncr.22530.
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Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy.原发性恶性肝上皮样血管内皮瘤:文献综述,重点关注手术治疗
Cancer. 2006 Nov 1;107(9):2108-21. doi: 10.1002/cncr.22225.
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Surgical treatment of adult primary hepatic sarcoma.成人原发性肝肉瘤的外科治疗
Br J Surg. 2000 Nov;87(11):1500-5. doi: 10.1046/j.1365-2168.2000.01564.x.
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Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma.上皮样血管内皮瘤:一种常被误诊为癌的血管肿瘤。
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现代原发性肝脏肉瘤:切除还是移植?

Primary liver sarcomas in the modern era: Resection or transplantation?

作者信息

Konstantinidis Ioannis T, Nota Carolijn, Jutric Zeljka, Ituarte Philip, Chow Warren, Chu Peiguo, Singh Gagandeep, Warner Susanne G, Melstrom Laleh G, Fong Yuman

机构信息

Department of Surgical Oncology, City of Hope National Medical Center, Duarte, California.

Department of Biostatistics, City of Hope National Medical Center, Duarte, California.

出版信息

J Surg Oncol. 2018 Apr;117(5):886-891. doi: 10.1002/jso.24979. Epub 2018 Jan 22.

DOI:10.1002/jso.24979
PMID:29355969
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5992101/
Abstract

BACKGROUND AND OBJECTIVES

Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients.

METHODS

We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS.

RESULTS

Of 237 patients identified, the majority were female (60.8%), with median age of 52 years. Histologies were: epithelioid hemangioendothelioma (n = 67), angiosarcoma (n = 64), leiomyosarcoma (n = 33), embryonal rhabdomyosarcoma (n = 31), carcinosarcoma (n = 16), giant cell sarcoma (n = 14), spindle cell sarcoma (n = 12). Ninety-seven (40.9%) patients underwent lobectomies or extended lobectomies, 41 patients (17.3%) underwent transplantation. Surgical margins were negative in 82.9%. Tumors were well differentiated in 11.3%. Histology type correlated with outcome with the best prognosis for epithelioid hemangioendothelioma (OS: not reached, similar for resection and transplantation) and the worst for angiosarcoma (OS:16.6 mo with resection; 6 mo with transplantation; P = 0.04). Resections with microscopically negative margins were associated with improved survival (58.7 vs 11.3 mo for positive margins; P < 0.001). Chemotherapy and radiation therapy were used in a minority of patients (32.9% and 4.3% respectively) with no improvement in outcomes.

CONCLUSIONS

Both hepatic resection and liver transplantation can be associated with long term survival for selected primary liver sarcomas such as epitheliod hemangioendotheliomas. Histology type and the ability to resect the tumor with negative margins correlate with outcomes and the decision to operate should be carefully weighed for subtypes with particularly dismal prognosis such as angiosarcomas.

摘要

背景与目的

原发性肝脏肉瘤(PLS)较为罕见。已发表的病例系列受限于患者数量较少。

方法

我们回顾了国家癌症数据库(2004 - 2014年)中接受PLS手术切除的患者。

结果

在237例确诊患者中,大多数为女性(60.8%),中位年龄为52岁。组织学类型包括:上皮样血管内皮瘤(n = 67)、血管肉瘤(n = 64)、平滑肌肉瘤(n = 33)、胚胎性横纹肌肉瘤(n = 31)、癌肉瘤(n = 16)、巨细胞肉瘤(n = 14)、梭形细胞肉瘤(n = 12)。97例(40.9%)患者接受了肝叶切除术或扩大肝叶切除术,41例(17.3%)患者接受了肝移植。手术切缘阴性率为82.9%。肿瘤高分化率为11.3%。组织学类型与预后相关,上皮样血管内皮瘤预后最佳(总生存期:未达到,手术切除和肝移植相似),血管肉瘤预后最差(总生存期:手术切除为16.6个月;肝移植为6个月;P = 0.04)。显微镜下切缘阴性的切除术与生存期改善相关(切缘阳性者为11.3个月,切缘阴性者为58.7个月;P < 0.001)。少数患者使用了化疗和放疗(分别为32.9%和4.3%),但预后无改善。

结论

对于某些特定的原发性肝脏肉瘤如上皮样血管内皮瘤,肝切除和肝移植均可带来长期生存。组织学类型以及切缘阴性切除肿瘤的能力与预后相关,对于预后特别差的亚型如血管肉瘤,手术决策应谨慎权衡。