Konstantinidis Ioannis T, Nota Carolijn, Jutric Zeljka, Ituarte Philip, Chow Warren, Chu Peiguo, Singh Gagandeep, Warner Susanne G, Melstrom Laleh G, Fong Yuman
Department of Surgical Oncology, City of Hope National Medical Center, Duarte, California.
Department of Biostatistics, City of Hope National Medical Center, Duarte, California.
J Surg Oncol. 2018 Apr;117(5):886-891. doi: 10.1002/jso.24979. Epub 2018 Jan 22.
Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients.
We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS.
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.
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.
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。
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。
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。
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。