Thum Chee, Hollowood Kevin, Birch Jeremy, Goodlad John R, Brenn Thomas
Department of Pathology, Western General Hospital, The University of Edinburgh, Edinburgh, UK.
J Cutan Pathol. 2011 Dec;38(12):954-60. doi: 10.1111/j.1600-0560.2011.01798.x.
Atypical fibroxanthoma (AFX) is a distinctive clinicopathologic entity presenting on sun-damaged skin of the elderly. Its behavior is benign if strict diagnostic criteria are applied. Tumors showing invasion of deeper structures or perineural/lymphovascular invasion are best regarded as undifferentiated pleomorphic sarcoma of the skin. The diagnosis requires immunohistochemical studies to exclude melanoma, squamous cell carcinoma, angiosarcoma and leiomyosarcoma.
Two AFX and one undifferentiated pleomorphic sarcoma showing aberrant expression of Melan-A were identified. Clinical data were obtained and histopathological features, immunohistochemical profile and electron microscopy were assessed.
All tumors arose on sun-damaged skin of elderly males. Two AFX showed pushing growth into superficial subcutis only. The undifferentiated pleomorphic sarcoma was characterized by infiltrative growth into galea as well as perineural invasion. Multifocal expression of Melan-A and MART-1 was largely limited to tumor giant cells in the absence of S100 or HMB-45 labeling. No melanosomes or premelanosomes were identified by electron microscopy.
Aberrant expression of Melan-A and MART-1 in AFX and undifferentiated pleomorphic sarcoma of the skin represents an important diagnostic pitfall with potential for misdiagnosis as melanoma.
非典型纤维黄色瘤(AFX)是一种独特的临床病理实体,出现在老年人受阳光损伤的皮肤上。如果应用严格的诊断标准,其行为是良性的。表现出侵犯更深层结构或神经周围/淋巴管侵犯的肿瘤最好视为皮肤未分化多形性肉瘤。诊断需要进行免疫组织化学研究以排除黑色素瘤、鳞状细胞癌、血管肉瘤和平滑肌肉瘤。
鉴定出两例AFX和一例显示Melan-A异常表达的未分化多形性肉瘤。获取临床数据并评估组织病理学特征、免疫组织化学谱和电子显微镜检查结果。
所有肿瘤均发生在老年男性受阳光损伤的皮肤上。两例AFX仅表现为向浅表皮下组织的推挤性生长。未分化多形性肉瘤的特征是向帽状腱膜浸润性生长以及神经周围侵犯。Melan-A和MART-1的多灶性表达在很大程度上仅限于肿瘤巨细胞,且无S100或HMB-45标记。电子显微镜检查未发现黑素小体或前黑素小体。
Melan-A和MART-1在AFX和皮肤未分化多形性肉瘤中的异常表达是一个重要的诊断陷阱,有可能误诊为黑色素瘤。