少见类型的肝脏原发性恶性肿瘤

Rare primary liver malignant tumor

目的提高对少见类型肝脏原发性恶性肿瘤的诊疗水平。方法收集我院经手术病理确诊的少见类型肝脏原发性恶性肿瘤40例,对各种类型肝脏原发性恶性肿瘤的临床特点进行回顾性总结。结果少见类型的原发性肝脏恶性肿瘤占同期所有原发性肝脏恶性肿瘤手术病例的0.74%(40/5487),包括12种病理类型:血管肉瘤6例,平滑肌肉瘤5例,恶性纤维组织细胞瘤5例,横纹肌肉瘤1例,胚胎性肉瘤1例,上皮样血管内皮瘤1例,淋巴瘤5例,囊腺癌4例,类癌3例,鳞癌2例,腺鳞癌3例,癌肉瘤4例。临床表现无症状者21例;右上腹痛者12例,其中肝肿瘤破裂1人;上腹不适者5例,黄疸2例,发热1例。实验室检查中32.5%HBsAg阳性(13/40),CT检查无1例直接确诊,其中3例肝脏淋巴瘤诊断为血管瘤或慢性炎症,3例肝肉瘤分别诊断为慢性炎症、血管瘤或囊腺瘤,2例鳞癌全部误诊为肝结核或肝囊肿,1例类癌诊断为纤维板层癌,1例囊腺癌诊断为肝囊肿,其余病例术前CT诊断均为肝脏恶性肿瘤。预后方面,4例肝淋巴瘤和2例肝囊腺癌生存期超过5年;肝肉瘤1年生存率超过50%,但3年生存率不超过25%;5例肝脏鳞癌、腺鳞癌病例均在术后1年内死亡。结论各种少见类型的肝脏原发性恶性肿瘤临床表现无特异性,实验室检查中亦无血清特异性肿瘤标志物,影像学与肝细胞肝癌和某些肝脏良性肿瘤很难鉴别,术前诊断十分困难。治疗仍以手术切除为主,预后以肝囊腺癌和肝淋巴瘤预后较好,以肝脏鳞癌和腺鳞癌预后最差。

Objective To improve the diagnosis and treatment of rare primary liver malignant tumor. Methods A retrospective analysis was made on 40 cases of rare liver malignant tumor,which were surgically resected in our hospital and had been confirmed by pathology. Results Rare liver malignant tumor accounted for 0. 740/oo in all primary liver malignant tumor （41）/5 487） in the same period, including 12 pathological kinds ： angiosarcoma （ 6 cases）, leiomyosarcoma （ 5 cases）, malignant fibrous histiocytoma （ 5 cases）, rhabdomyosarcoma （ 1 case）, undifferentiated embryonal sarcoma （ 1 case）, cystadenocarcinoma（4 cases）, epithelioid hemangioendothelioma （ 1 case）, lymphoma （ 5 cases）, squamous cell cancer （ 2 cases ）, adenosquamous cancer （ 3 cases ）, carnoid cancer （ 3 cases ）, carcinosarcoma （4 cases）. Twenty one of them had no clinical presentation, 12 of them complained right upper quadrant pain,2 of them presented jaundice,l of them complained fever. About 32.5% of them was positive of HBsAg （13/40）,1 case of rhabdomyosarcoma AFP was elevated, 4 cases of liver cystadenocarcinoma or squmous cell cancer or adenosquamous carcinoma CA199 were elevated . No patients was diagnosed directedly by image before operation. Three cases of primary hepaticlymohoma patients were diagonsed hemangioma or chronic infection. Three cases of primary liver sracoma were diagnosed chronic infection, hemangioma or cystadenoma. Two cases primary squamous cell cancer was diago nosed liver tuberculosis. One case of carnoid was diagnosed fibrous luminar malignant tumor. One cystadenocarcinoma was diagnosed liver cyst. Other patients was diagnosed liver maligant tumor. As toprognosis,4 cases of primary liver lymphoma and 2 cases liver cystadenocarcinoma survived over five years. Fifty percent of primary liver sarcoma survived over one year. but at most 25% primary liver sarcoma survived over 3 years. All liver squamous cell cancer and liver adnenosquamous carcinoma died in one year after surgery. Conclusions Rare primary liver malignant tumor has no specific clinical presentation or serum tumor markers. It was difficult to differentiated between them and other liver tumor such as hepatocellular carcinoma or some benign liver tumor, so they are very difficult to be diagnosed before operation. The mainstay therpy is surgical resection, and the prognosis of liver cystadenocarcinoma and primary hepatic lymphoma is better, while the prognosis of liver squamous cell carcinoma and adenosquamous carcinoma is the worst.