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Myofibromas with atypical features: expanding the morphologic spectrum of a benign entity.

伴有非典型特征的肌纤维瘤:一种良性疾病扩展的形态学谱系

Linos K,Carter JM,Gardner JM,Folpe AL,Weiss SW,Edgar MA

Abstract

Typical myofibromas are biphasic tumors composed of a central zone of immature spindled to rounded cells arranged in a pericytic pattern and a peripheral zone of myoid nodules. Central necrosis is occasionally seen. A small but undefined subset of myofibromas displays atypical features that may lead to a misdiagnosis of sarcoma. To more completely characterize these tumors and define their behavior, we analyzed our experience with myofibromas having 1 or more atypical features including hypercellularity, absent or inconspicuous, poorly demarcated myoid nodules, infiltrative growth pattern, and perineural invasion. Of 266 cases of myofibromas, 24 cases were retrieved on the basis of pathology reports in which atypical features were mentioned. The tumors presented in 16 male and 8 female individuals (mean age 17 y; range, 2 wk to 62 y) as masses of variable size (mean 3.0 cm; range, 1.5 to 6.5 cm). Fourteen cases arose on the head and neck and 10 cases on the limbs. The referring or suspected diagnosis was sarcoma in 8 cases. The tumors were typically more cellular than ordinary myofibroma with levels of cellularity similar to that expected in fibrosarcoma (22/24). In addition, they displayed inconspicuous, loosely cohesive (22/24) or absent myoid nodules (2/24), infiltrating borders (19/24), intravascular growth (5/24), and perineural invasion/nerve entrapment (6/24). The mean mitotic rate was 5 mitoses/10 high-power fields, but no tumor showed significant cytologic atypia. The tumors were positive for actins (11/11) and CD34 (2/8). Follow-up in 14 patients revealed no distant metastases. We conclude that a small subset of myofibromas shows atypical features that complicate the diagnosis but do not adversely affect outcome.

摘要

典型的肌纤维瘤是一种双相结构肿瘤,中央区为不成熟的梭形、圆形细胞呈血管外皮瘤样排列,外周区为肌样结节。有时可见中央坏死。有一小部分肌纤维瘤具有非典型性特点,它们的临床病理特点尚未明确界定,容易误诊为肉瘤。为了更完整地描述这些肿瘤和定义它们的行为,我们分析了所遇到的具有1个或更多非典型特征的肌纤维瘤,这些非典型特征包括富于细胞、界不清的肌样结节缺失或不明显,浸润性生长模式和神经浸润。在266例肌纤维瘤中,有24例在病理报告中提到了具有非典型特征。这些肿瘤包括16例男性,8例女性,年龄2周到62岁,平均17岁。肿块大小1.5到6.5cm,平均3.0cm。14例发生在头颈部,10例发生在肢体。8例倾向或可疑的诊断是肉瘤。这些肿瘤比普通的肌纤维瘤更富于细胞,与纤维肉瘤的细胞丰富度相似(22/24)。另外,它们具有不明显的,粘附性差(22/24)的肌样结节,或缺乏肌样结节(2/24),浸润性生长(19/24),血管内生长(5/24),神经周围浸润/神经挤压(6/24)。平均核分裂为5/10HPF,但没有肿瘤有明显的细胞非典型性。肿瘤细胞表达actins(11/11)和CD34(2/8)。14例患者随访无远处转移。我们认为具有非典型性的一小部分肌纤维瘤虽然使诊断复杂化,但对预后并无不良影响。


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