Primary hepatic malignant fibrous histiocytoma mimicking cystadenocarcinoma: a case report.
Primary hepatic malignant fibrous histiocytoma mimicking cystadenocarcinoma: a case report.
复制标题
原发性肝恶性纤维组织细胞瘤模仿囊腺癌：病例报告。
DOI：
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发表时间：
2006
影响因子：
3.3
通讯作者：
W. Cong
中科院分区：
文献类型：
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作者：
Guang;Meng‐chao Wu;Jia‐he Yang;Shuqun Cheng;Nan Li;Kai Liu;Binghua Dai;W. Cong
背景
恶性纤维组织细胞瘤是最常见的软组织肉瘤，好发于四肢，但较少见于腹膜后间隙、腹腔或其他部位。原发性肝脏恶性纤维组织细胞瘤是非常罕见的，迄今为止在英文文献中仅报道了28例。
方法
本文报告一例原发性肝脏恶性纤维组织细胞瘤的临床表现、诊断、治疗及预后。
结果
一位50岁的男性在肝脏左叶有一个巨大的多囊性肿块病变，无法通过剖腹手术进行手术。术后病理检查证实为恶性纤维组织细胞瘤，形态多样。肿瘤迅速发展，患者在手术后2个月死于肝功能衰竭。
结论
摘要肝脏原发性恶性纤维组织细胞瘤因其罕见及无特异性的表现而在晚期才被诊断。如果可行，手术切除是第一步治疗。原发性肝脏恶性纤维组织细胞瘤的预后很差，据报道中位生存期为3个月。外科医生应警惕肝脏中存在这种类型的软组织肿瘤。
BACKGROUND
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.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.