首页 > 期刊杂志 > 正文

Myxoinflammatory fibroblastic sarcoma: a clinicopathologic analysis of 104 cases, with emphasis on predictors of outcome.

粘液样炎性纤维母细胞肉瘤预后因素:104例临床病理分析

Laskin WB,Fetsch JF,Miettinen M

Abstract

In this study, we examine the clinicopathologic features of 104 cases of myxoinflammatory fibroblastic sarcoma (MIFS), a low-grade, inflammatory fibromyxoid tumor with a predilection to distal extremity soft tissue, and attempt to identify factors predictive of aggressive behavior. The study cohort consisted of 49 male and 55 female patients ranging in age from 17 to 83 (mean, 42; median, 39) years. The tumor arose primarily on the dorsal aspect of the distal extremities as a solitary and usually painless mass. Tumors ranged in size from 0.5 to 15 (mean, 3.2; median; 2.4) cm. Microscopically, tumors consisted of variably cellular and inflamed fibromyxoid tissue growing as a lobulated mass or as multiple nodules within subcutaneous tissue or along tendinofascial planes. Tumor cells ranged from plump spindled to more epithelioid cells with enlarged, vesicular nuclei. Characteristic of the process was a strikingly bizarre cell with an inclusion body-like nucleolus (85% of cases) and/or a smudgy hyperchromatic nucleus (51%) present in all but 7 cases. The mitotic rate per 50 high-power field ranged from 0 to 13 (mean, 2,9; median, 2) mitoses. Twenty-two tumors demonstrated 1 or more of the following atypical features: (1) foci with complex sarcoma-like vasculature; (2) hypercellular areas; and (3) increased mitotic activity or atypical mitotic figures. Immunohistochemically, tumor cells demonstrated immunoreactivity for vimentin (100%), D2-40 (86%), CD34 (50%), keratin(s) (33%), CD68 (27%), actin(s) (26%), desmin (9%), S-100 protein (7%), and epithelial membrane antigen (6%). Thirty of 59 patients (51%) with follow-up data suffered (at least) 1 local recurrence, and 1 patient developed metastatic disease after multiple local recurrences. Completeness of initial surgical excision was the only clinicopathologic parameter that statistically correlated with a lower incidence of recurrence (P=0.004). Histologically atypical MIFS recurred more often than conventional tumors (67% vs. 47%), but the difference was not statistically significant (P=0.35). Our study shows that histologic features often associated with more aggressive sarcomas do not substantially impact the morbidity of MIFS, and complete surgical excision provides the best chance for disease-free survival.

摘要

我们研究了104例粘液样炎性纤维母细胞肉瘤(MIFS)——一种好发于远端肢体软组织的低级别炎性纤维黏液肿瘤,拟明确能预测其侵袭行为的因素。研究对象包括49名男性55名女性患者,年龄从17岁到83岁(平均年龄42岁,中位年龄39岁)。肿瘤主要表现为四肢远端背侧一个孤立的,通常无痛性的肿块。肿瘤大小从0.5cm?15cm(平均3.2cm,中位数2.4cm)。镜下,肿瘤主要由丰富的细胞和炎性纤维粘液组织形成一个位于皮下或沿筋膜分布的分叶状多结节肿物。肿瘤细胞自胖梭形细胞或具有更大泡状核的上皮样细胞而不等。肿瘤的特点是具有含包涵体样核仁(85%的病例)和/或污浊、深染细胞核(51%)的怪异细胞——但有7例未发现此类细胞。每50高倍视野下肿瘤细胞有丝分裂数为0?13(平均2,9,中位数2)个。有22例肿瘤表现出1个及以上的下述非典型特征:(1)局灶具有复杂肉瘤样脉管系统;(2)富于细胞区;(3)有丝分裂增多或不典型核分裂。免疫组化上肿瘤细胞检测出波形蛋白(100%),D2-40(86%),CD34(50%),角蛋白(S)(33%),CD68(27%),肌动蛋白(S)(26%),结蛋白(9%),S-100蛋白(7%)和上皮细胞膜抗原(6%)。随访的59例中有30例(51%)出现至少1处的局部复发,,有1例患者在多处局部复发后出现转移。首次手术完整切除肿瘤,是与低复发率有统计学相关的唯一因素(P=0.004)。组织学上非典型MIFS往往比典型的肿瘤复发率高(67%对47%),但差异无统计学显著性(P=0.35)。我们的研究表明,看起来更具侵袭性的肉瘤相关组织学特征基本不影响MIFS的发病率,彻底的手术切除则为无病生存提供了最佳机会。

full text

我要评论

0条评论