Feng Long-Hai, Dong Hui, Zhu Yu-Yao, Cong Wen-Ming
Department of Pathology, Eastern Hepatobiliary Surgery Hospital, The Second Military Medical University, 225 Changhai Road, Yangpu, Shanghai 200438, China.
Department of Pathology, Eastern Hepatobiliary Surgery Hospital, The Second Military Medical University, 225 Changhai Road, Yangpu, Shanghai 200438, China.
Pathol Res Pract. 2015 Dec;211(12):911-7. doi: 10.1016/j.prp.2015.09.004. Epub 2015 Sep 10.
Primary hepatic solitary fibrous tumor is a rare neoplasm that originates in the submesothelial tissue of the liver and is frequently misdiagnosed because of its rarity and unfamiliar characteristics.
To analyze, summarize and update the clinical and pathological features of primary hepatic solitary fibrous tumor.
We systematically extract the clinical data of 4 cases from the relevant medical records, analyze the macroscopic, histological and immunohistochemical features and review the 59 previously reported cases in the English literatures.
The patients' mean age 50.75 years (range, 49-52 years), and the gender ratio was 1:1. The tumors ranged in size from 2.3 to 12.0cm (average diameter, 7.85cm). The tumors were composed of spindle cells with oval, fusiform or banded nuclei that were arranged in bundled, storiform or peculiar random patterns. Mitosis and hemangiopericytoma-like vessels were occasionally observed. Immunohistochemically, three cases were positive for Signal transduction and activator of transcription 6, cluster of differentiation 34, B-cell lymphoma-2 and vimentin but were negative for nervous, muscular and hepatocellular markers. Hepatic lobectomy was performed in all cases, and one patient received adjuvant chemotherapy simultaneously. One patient suffered two recurrences without metastasis, and the remaining patients experienced favorable outcomes.
A consensus on the essential and definite diagnostic criteria for primary hepatic solitary fibrous tumor must be reached in a timely manner. Signal transduction and activator of transcription 6 is a highly sensitive and specific immunohistochemical marker for primary hepatic solitary fibrous tumor.
原发性肝脏孤立性纤维瘤是一种罕见的肿瘤,起源于肝脏的间皮下组织,因其罕见性和不熟悉的特征而经常被误诊。
分析、总结和更新原发性肝脏孤立性纤维瘤的临床和病理特征。
我们从相关病历中系统提取4例患者的临床资料,分析其大体、组织学和免疫组化特征,并复习英文文献中先前报道的59例病例。
患者的平均年龄为50.75岁(范围49 - 52岁),性别比为1:1。肿瘤大小为2.3至12.0厘米(平均直径7.85厘米)。肿瘤由梭形细胞组成,细胞核呈椭圆形、梭形或带状,排列成束状、席纹状或特殊的随机模式。偶尔可见核分裂象和类似血管外皮细胞瘤的血管。免疫组化方面,3例信号转导及转录激活因子6、分化簇34、B细胞淋巴瘤 - 2和波形蛋白呈阳性,但神经、肌肉和肝细胞标志物呈阴性。所有病例均行肝叶切除术,1例患者同时接受辅助化疗。1例患者复发2次无转移,其余患者预后良好。
必须及时就原发性肝脏孤立性纤维瘤的基本和明确诊断标准达成共识。信号转导及转录激活因子6是原发性肝脏孤立性纤维瘤高度敏感和特异的免疫组化标志物。