Ding Guang-Hui, Wu Meng-Chao, Yang Jia-He, Cheng Shu-Qun, Li Nan, Liu Kai, Dai Bing-Hua, Cong Wen-Ming
Department of Comprehensive Treatment, Eastern Hepatobiliary Surgery Hospital, Second Military Medical University, Shanghai 200438, China.
Hepatobiliary Pancreat Dis Int. 2006 Nov;5(4):620-3.
Malignant fibrous histiocytoma is the most common sarcoma of soft tissue, which occurs usually in the extremities, but less common in the retroperitoneal space, abdominal cavity or other sites. Primary malignant fibrous histiocytoma of the liver is extremely rare; only 28 cases have been reported to date in the English literature.
In this report, a case of primary malignant fibrous histiocytoma of the liver was described in terms of clinical presentations, diagnosis and treatment and outcome.
A 50-year-old man had a large multicystic-mass lesion in the left lobe of the liver, which was inoperable by laparotomy. Pathological examination of biopsy specimen after operation confirmed a malignant fibrous histiocytoma of storiform-pleomorphic type. The tumor developed rapidly, and the patient died of hepatic failure 2 months after the surgery.
Primary malignant fibrous histiocytoma of the liver is diagnosed in late stage because of its rarity and non-specific presentations. Surgical resection, if feasible, is the first step treatment. The prognosis of primary malignant fibrous histiocytoma of the liver is grim with a median survival of 3 months as reported. Surgeons should be alert to the existence of this type of soft tissue tumor in the liver.
恶性纤维组织细胞瘤是最常见的软组织肉瘤，通常发生于四肢，但在腹膜后间隙、腹腔或其他部位较少见。原发性肝脏恶性纤维组织细胞瘤极为罕见；迄今为止，英文文献中仅报道了28例。
在本报告中，描述了1例原发性肝脏恶性纤维组织细胞瘤的临床表现、诊断、治疗及转归。
一名50岁男性肝脏左叶有一个巨大的多囊性肿块病变，经剖腹手术无法切除。术后活检标本的病理检查证实为席纹状多形型恶性纤维组织细胞瘤。肿瘤发展迅速，患者术后2个月死于肝衰竭。
原发性肝脏恶性纤维组织细胞瘤因其罕见性及非特异性表现而在晚期才得以诊断。若可行，手术切除是首要治疗措施。据报道，原发性肝脏恶性纤维组织细胞瘤的预后严峻，中位生存期为3个月。外科医生应警惕肝脏中存在此类软组织肿瘤。
