原发性肝脏肉瘤样癌临床特征及外科治疗

Analysis of clinical features and surgery treatment of 7 cases of primary liver sarcomatoid carcinoma

目的:探讨原发性肝脏肉瘤样癌(SHC)的临床特征、外科治疗及预后,提高临床医师对此病的认识。方法:收集我院收治的7例病理证实为SHC患者的临床资料,对其临床症状、影像学表现、手术方法、病理特征及预后进行回顾性分析。结果:SHC患者临床上主要表现为腹痛、腹胀、乏力、食欲下降或无症状;影像学表现不典型,CT表现具有癌及肉瘤双重特征,增强有不同程度强化;7例患者中4例行根治性肿瘤切除,2例姑息性切除,1例单纯癌肿破裂修补;病理镜下见肿瘤明确的癌和肉瘤样双相成分,肉瘤样变区癌细胞形态多样;免疫组织化学染色结果显示,上皮样标志物CK阳性7例、EMA阳性5例、MSA阳性1例、CK7阳性2例、Hep Par-1阳性3例,间叶组织标志物Vimentin阳性6例、DES阳性1例。随访期内2例无瘤存活,2例复发、带瘤存活,3例死亡。结论:SHC是一种极其罕见的恶性肿瘤,是癌的化生变形,无特异性临床表现及影像学特征,细胞病理特征复杂多样,分化较差,诊断主要依据免疫组织化学染色。手术切除肿瘤是主要治疗手段,其对放化疗不敏感,预后极差。

Objective： To explore the clinical and pathological features of primary liver sarcomatoid carci-noma and to discuss its diagnosis, the operative method and prognosis. Methods： The clinical data of 7 patients with pathologically proven sarcomatoid carcinoma of the liver were retrospectively reviewed in our hospital from February 2004. Results： Three cases showed right upper abdomen pain, abdomen distension in 1 case, 1 case of anorexia and fatigue, 2 cases were asymptomatic. The CT appearances of sarcomatoid carcinoma had both characteristics of hepatic cancer and carcinoma CT signs could show malignant characlteristics of lesion.Varied degrees of contrast enhancement could be visualized. All the patients were undergone surgery. The tumor was composition with cancer and sarcoma-like areas, and the tumor cells of sarcomatoid areas were very complex. Immunohistochemical positive for CK （7）, EMA （5）, MSA （1）, CK7 （2）, HepPar-1 （3） and Vimentin （6）, DES （1）. The length of follow-up ranged from 17 days to 35 months during which time 2 patients with disease free, 2 survived as tumer carriers and 3 patients died. Conclusion： Liver sarcomatoid carcinoma is a kind of extremely rare malignant tumor, with untypical clinical symptoms, non-specific imaging feature, tumor cells with complex and diverse pathological features, diagnosed according to immtmohistochemistry. Surgical resection is the main treatment, insensitive to radiotherapy and chemotherapy, with worse prognosis.