Department of Dermatology, Nara Prefecture General Medical Center, Nara, Japan.
Department of Dermatology, Nara Medical University School of Medicine, Kashihara, Japan.
Am J Dermatopathol. 2024 Oct 1;46(10):675-678. doi: 10.1097/DAD.0000000000002773. Epub 2024 Jun 28.
Dermatofibrosarcoma protuberans (DFSP) is a neoplasm of intermediate malignancy with high local recurrence rates. The sclerosing variant is characterized by the presence of sclerotic areas in more than 50% of tumors and is rarely reported. In this report, we describe a case of sclerosing DFSP with areas histopathologically resembling sclerotic fibroma, where the initial biopsy tissue presented a diagnostic challenge. A 77-year-old man presented with a 2-cm firm, erythematous nodule on the chest. A punch biopsy revealed plywood-like sclerosis and spindle cells with a vaguely storiform pattern. The tumor cells were positive for CD34. Sclerotic fibroma and DFSP were considered differential diagnoses. Subsequent excisional biopsy revealed that the tumor comprised 3 different histopathological areas: classic DFSP, sclerotic fibroma-like, and giant cell fibroblastoma-like. This report highlights the importance of reevaluating the clinical context and excision for further characterization.
隆突性皮肤纤维肉瘤（DFSP）是一种中度恶性肿瘤，局部复发率高。硬化型的特征是超过 50%的肿瘤存在硬化区，很少有报道。在本报告中，我们描述了一例硬化型 DFSP 病例，其组织病理学上存在类似于硬化性纤维瘤的区域，最初的活检组织具有诊断挑战性。一名 77 岁男性胸部出现一个 2 厘米大小的坚实、红斑性结节。活检显示板层样硬化和梭形细胞，呈模糊的席纹状模式。肿瘤细胞 CD34 阳性。硬化性纤维瘤和 DFSP 被认为是鉴别诊断。随后的切除活检显示肿瘤由 3 个不同的组织病理学区域组成：经典 DFSP、硬化性纤维瘤样和巨细胞成纤维细胞瘤样。本报告强调了重新评估临床背景和切除以进一步特征化的重要性。
