Meng Guo-zhao, Zhang Hong-ying, Bu Hong, Zhang Xian-liang, Pang Zong-guo, Ke Qi, Liu Xi, Yang Guo
Department of Pathology and Laboratory of Pathology, West China Hospital, Sichuan University, Chengdu 610041, China.
Chin Med J (Engl). 2007 Mar 5;120(5):363-9.
Myofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations.
The paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student's t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas.
Histologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform, tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophilic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin (18/20), muscle specific actin (16/20), fibronectin (20/20) and desmin (2/20). Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease.
Myofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.
肌纤维母细胞肉瘤曾是一种存在争议的肿瘤。本研究调查了20例发生于不同部位的肌纤维母细胞肉瘤的临床病理特征。
对20例肌纤维母细胞肉瘤患者的石蜡包埋组织样本进行免疫组织化学染色,并对其中5例进行电镜检查。采用Student's t检验分析1级和2级肌纤维母细胞肉瘤之间Ki-67标记指数的差异。
组织学上,肿瘤由具有嗜酸性胞质的细长梭形细胞组成,呈梭形、逐渐变细、波浪状或饱满的卵圆形;核呈泡状,有小的中央嗜酸性核仁。免疫组织化学显示,肿瘤细胞表达平滑肌肌动蛋白(18/20)、肌肉特异性肌动蛋白(16/20)、纤连蛋白(20/20)和结蛋白(2/20)。超微结构上,肿瘤细胞胞质内可见丰富的粗面内质网和纵向排列的细肌丝,有局灶性致密物。对19例患者的临床随访显示,2例在初次手术后6个月至4年出现局部复发和远处转移。9例在初次切除后17至46个月出现局部复发,9例存活且无疾病证据。
肌纤维母细胞肉瘤具有多样的组织学表现,容易被误诊为良性肿瘤。肌纤维母细胞肉瘤是具有局部破坏性的病变,复发频繁,且可能发生远处转移。