Pinson C W, Lopez R R, Ivancev K, Ireland K, Sawyers J L
Department of Surgery, Vanderbilt University School of Medicine, Nashville, Tenn.
South Med J. 1994 Mar;87(3):384-91. doi: 10.1097/00007611-199403000-00017.
Four patients had resection for primary hepatic sarcoma: one with malignant fibrous histiocytoma (MFH), two with poorly differentiated fibrosarcoma, and one with leiomyosarcoma. Age ranged from 40 to 69 years. One patient had a cousin and a grandmother who had died of hepatic tumors. At presentation, all patients had pain; one had tumor rupture, and one had mental changes and hypoglycemia. None had hepatitis or cirrhosis. Results of laboratory evaluation were nonspecific, including normal carcinoembryonic antigen and alpha-fetoprotein levels. Computed tomography showed hypodense masses with enhancement. Angiography showed a hypervascular mass in three patients and an avascular mass in the patient with MFH. Despite large tumors (8 to 32 cm), portal and hepatic veins were not invaded. The pattern of vascularization and lack of venous invasion helps differentiate primary hepatic sarcomas from hepatocellular carcinoma, especially in noncirrhotic patients. All patients had extensive hepatic resections, with one operative death. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. The patient with MFH died with recurrence at 10 1/2 months. The patient with the ruptured fibrosarcoma had a second resection and chemotherapy, but died with recurrence at 3 years. The patient with the leiomyosarcoma had a second resection and was disease free at 4 years. Resection of primary hepatic sarcoma is warranted, with potential survival measured in years.
4例患者接受了原发性肝肉瘤切除术:1例为恶性纤维组织细胞瘤(MFH),2例为低分化纤维肉瘤,1例为平滑肌肉瘤。年龄在40至69岁之间。1例患者有一位死于肝肿瘤的堂兄和一位祖母。就诊时,所有患者均有疼痛;1例发生肿瘤破裂,1例有精神改变和低血糖。均无肝炎或肝硬化。实验室检查结果无特异性,包括癌胚抗原和甲胎蛋白水平正常。计算机断层扫描显示低密度肿块并强化。血管造影显示3例患者为高血运肿块,MFH患者为无血运肿块。尽管肿瘤较大(8至32 cm),但门静脉和肝静脉未受侵犯。血管化模式和无静脉侵犯有助于将原发性肝肉瘤与肝细胞癌区分开来,尤其是在非肝硬化患者中。所有患者均接受了广泛的肝切除术,1例手术死亡。肿瘤的免疫组织化学染色波形蛋白阳性,但上皮标记物阴性,从而将这些病变与其他肝肿瘤区分开来。MFH患者在10个半月时死于复发。纤维肉瘤破裂的患者接受了二次切除和化疗,但在3年时死于复发。平滑肌肉瘤患者接受了二次切除,4年时无疾病。原发性肝肉瘤的切除是必要的,潜在生存期可达数年。