Department of Hepatobiliary and Splenic Surgery, Shengjing Hospital of China Medical University, Shenyang, Liaoning Province, China.
World J Surg Oncol. 2012 Jan 5;10:2. doi: 10.1186/1477-7819-10-2.
A malignant fibrous histiocytoma is a soft tissue tumor that most commonly occurs in the extremities, but rarely involves the liver. The clinical characteristics and therapeutic experiences of primary hepatic malignant fibrous histiocytoma are still limited.
Two cases of primary hepatic malignant fibrous histiocytoma were analyzed retrospectively, and all the literature concerning primary hepatic malignant fibrous histiocytoma was analyzed.
In China, a total of 76 cases had been reported, among which 50 were men, with a male to female ratio of 1.9:1. Mean age of the patients was 51.0 years old, and more than 85 percent were older than 40 years. 82.9 percent (63/76) of hepatic MFH were solitary lesions, with tumor size ranging from 2.5 to 23.5 cm (average 10.3 cm). Major clinical presentation (78.4%) was abdominal pain or discomfort, accompanied with some other non-specific symptoms such as malaise, anorexia, weight loss, jaundice and fever, and small cases (14.9%) were asymptomatic. Computed tomography and ultrasound usually revealed the location of lesions. The rate of pre-operative misdiagnosis was extremely high, and 14.9 percent of patients were even misdiagnosed as a benign liver cyst, liver abscess or hematoma. Integrated resection was performed among the most cases (49/68), among which only a few ones (12 cases) were introduced to have no recurrence or metastasis or be still alive with no detail information provided, while among the cases with palliative operation or only a biopsy, the cases that were followed-up all died.
Hepatic malignant fibrous histiocytoma is a rare malignant mesenchymal tumor. The variable features of clinical presentations and images make the diagnosis difficult. Though the prognosis of primary hepatic malignant fibrous histiocytoma was rather poor, integrated resection might provide a few cases a good opportunity for surviving, suggesting that surgery might be an effective treatment.
恶性纤维组织细胞瘤是一种软组织肿瘤，最常发生于四肢，但很少累及肝脏。原发性肝恶性纤维组织细胞瘤的临床特征和治疗经验仍有限。
回顾性分析 2 例原发性肝恶性纤维组织细胞瘤病例，并分析所有关于原发性肝恶性纤维组织细胞瘤的文献。
在中国，共报道了 76 例病例，其中男性 50 例，男女比例为 1.9:1。患者平均年龄为 51.0 岁，超过 85%的患者年龄大于 40 岁。82.9%（63/76）的肝 MFH 为单发病变，肿瘤大小为 2.5 至 23.5cm（平均 10.3cm）。主要临床表现（78.4%）为腹痛或不适，伴有一些其他非特异性症状，如乏力、食欲不振、体重减轻、黄疸和发热，少数病例（14.9%）无症状。计算机断层扫描和超声通常能显示病变位置。术前误诊率极高，14.9%的患者甚至被误诊为良性肝囊肿、肝脓肿或血肿。大多数病例（49/68）进行了综合切除，其中只有少数（12 例）病例没有复发或转移，仍存活且没有提供详细信息，而在姑息性手术或仅行活检的病例中，所有随访的病例均死亡。
肝恶性纤维组织细胞瘤是一种罕见的恶性间叶瘤。临床表现和影像学特征的多样性使诊断困难。虽然原发性肝恶性纤维组织细胞瘤的预后较差，但综合切除可能为少数病例提供良好的生存机会，提示手术可能是一种有效的治疗方法。
