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儿科患者的皮肤肌纤维瘤:临床病理特征及鉴别诊断

Dermatomyofibromas presenting in pediatric patients: clinicopathologic characteristics and differential diagnosis.

作者信息

Tardío Juan C, Azorín Daniel, Hernández-Núñez Almudena, Guzmán Ana, Torrelo Antonio, Herráiz Marisa, Espinosa Pablo, Moreno Amalia, Granados Rosario, Colmenero Isabel

机构信息

Department of Pathology, Hospital Universitario de Fuenlabrada, Madrid, Spain.

出版信息

J Cutan Pathol. 2011 Dec;38(12):967-72. doi: 10.1111/j.1600-0560.2011.01739.x. Epub 2011 Jul 14.

Abstract

Dermatomyofibroma represents a rare benign fibroblastic/ myofibroblastic cutaneous tumor that mostly occurs in young adult women. It has been seldom reported in pediatric patients. In this analysis, the clinical, histopathological and immunohistochemical findings of 12 dermatomyofibromas occurring in patients up to 16 years of age are compared with those reported in adults. Six patients were male and six were female. Nine lesions were located on the neck, two on the back and one involved the chest. The usual presentation was as an asymptomatic plaque composed of bland spindled cells arranged in dermal fascicles that were oriented parallel to the epidermis. Immunohistochemically, the lesional cells expressed calponin in 11 cases, smooth muscle actin in six and muscle-specific actin in three. In contrast to prior reports from adults, dermatomyofibromas in pediatric patients do not show a female predilection. In addition, they are mostly located on the neck (56%), while in adults the most frequent location is the shoulder (35%). Dermatomyofibromas seem to stabilize after an initial period of enlargement. Punch biopsy and clinical follow up could be an alternative approach to the surgical excision in some cases of dermatomyofibroma, particularly in instances in which surgery might inflict cosmetic defects.

摘要

皮肤肌纤维瘤是一种罕见的良性成纤维细胞/肌成纤维细胞性皮肤肿瘤,多见于年轻成年女性。儿科患者中鲜有报道。在本分析中,将16岁及以下患者发生的12例皮肤肌纤维瘤的临床、组织病理学和免疫组化结果与成人报道的结果进行了比较。6例为男性,6例为女性。9个病灶位于颈部,2个位于背部,1个累及胸部。通常表现为无症状斑块,由排列在真皮束中的温和梭形细胞组成,这些真皮束与表皮平行排列。免疫组化显示,11例病变细胞表达钙调蛋白,6例表达平滑肌肌动蛋白,3例表达肌肉特异性肌动蛋白。与成人先前的报道不同,儿科患者的皮肤肌纤维瘤没有女性偏好。此外,它们大多位于颈部(56%),而成人最常见的部位是肩部(35%)。皮肤肌纤维瘤在最初的增大期后似乎会稳定下来。在某些皮肤肌纤维瘤病例中,特别是在手术可能造成美容缺陷的情况下,钻孔活检和临床随访可能是手术切除的替代方法。

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