When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

A. Dumitru, A. Ciongariu, Cătălin Aliuș, D. Dumitrescu, Sergiu-Andrei Iordache, B. Șerban, D. Ionescu, M. Costache, M. Sajin, D. Țăpoi
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Abstract

Abstract Bednar tumor is a rare cutaneous neoplasm, regarded as a variant of dermatofibrosarcoma protuberans with melanotic colonization and it usually affects young and middle-aged adults or children. This lesion is considered a low-grade malignant tumor, which can be associated with multiple local recurrences after surgical excision. Although a rare phenomenon, these lesions may undergo fibrosarcomatous transformation, which implies a poorer prognosis of the disease, as the tumor has a more locally aggressive behavior and patients might also develop distant metastases. We present the case of a 53-year-old female patient, with no significant medical history, who presented with a subcutaneous nodule on her upper back, for which a wide surgical excision was performed. The gross examination of the specimen showed a solitary protuberant grey-white nodule with a bluish shade and flecked with pigment. The microscopic examination revealed a malignant proliferation with a predominantly fascicular growth pattern, composed of spindle cells with highly pleomorphic nuclei and high mitotic rate, as well as the presence of dendritic cells with abundant melanin. Upon immunohistochemical analysis, the proliferation showed negative staining for CD 34 and AE 1/3, whereas the scattered dendritic cells stained positive with S100 protein. Ki 67 was positive in 15% of the tumor cells and the absence of p53 expression was noted. Thus, the diagnosis of Bednar tumor with fibrosarcomatous transformation was established. The aim of this paper was to gain further knowledge about the histopathological and immunohistochemical features, as well as about the treatment of Bednar tumor, especially considering its rarity.
当一个旧的色素病变变得严重:低级别皮肤恶性肿瘤的诊断问题-贝德纳肿瘤伴纤维肉瘤转化
Bednar肿瘤是一种罕见的皮肤肿瘤,被认为是黑色素定植的皮肤纤维隆突肉瘤的一种变体,常见于中青年或儿童。这种病变被认为是一种低级别恶性肿瘤,在手术切除后可伴有多发局部复发。虽然这是一种罕见的现象,但这些病变可能会发生纤维肉瘤转化,这意味着该疾病的预后较差,因为肿瘤具有更局部的侵袭性行为,患者也可能发生远处转移。我们报告一例53岁的女性患者,没有明显的病史,她在上背部出现皮下结节,为此进行了大面积手术切除。大体检查显示一个孤立的灰白色突起结节,带蓝色阴影,有色素斑点。显微镜检查显示恶性增生以束状生长为主,由梭形细胞组成,细胞核高度多形性,有丝分裂率高,树突状细胞含有丰富的黑色素。经免疫组化分析,增殖细胞cd34和AE 1/3呈阴性染色,而分散的树突状细胞S100蛋白呈阳性染色。Ki 67在15%的肿瘤细胞中呈阳性,p53表达缺失。由此,确立了Bednar肿瘤伴纤维肉瘤转化的诊断。本文的目的是进一步了解Bednar肿瘤的组织病理学和免疫组织化学特征,以及治疗方法,特别是考虑到它的罕见性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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