Ledniczky György, Bognár Gábor, Diczházi Csaba, Bereczky Bíborka, Barabás Loránd, Ondrejka Pál
Semmelweis Egyetem, Altalános Orvostudományi Kar II sz Sebészeti Klinika, Budapest.
Magy Seb. 2009 Feb;62(1):27-9. doi: 10.1556/MaSeb.62.2009.1.7.
Dermatofibrosarcoma protuberans (DFSP) is a morphologically distinct dermal/subcutaneous fibrohystiocytic neoplasm seen mainly on the trunk and the proximal extremities, which generally is regarded as a low-grade or "borderline" neoplasm. Dermatofibrosarcoma protuberans is characterized clinically by locally aggressive growth and a high rate of local recurrence, but distant metastases and tumor-related deaths are very rare. DFSP in the breast is extremely uncommon. Immunohistochemically, DFSP is characterized by positive staining for vimentin and CD34. We present a case of a 65 years old female patient with a giant DFSP developed in the sight of several excised fibroadenoma and neurofibroma treated by radical mastectomy and axillary block-dissection. Perioperative hystological and immunohistochemical images demonstrate the characteristics of the giant nonphylloid sarcoma.
隆突性皮肤纤维肉瘤（DFSP）是一种形态学上独特的真皮/皮下纤维组织细胞性肿瘤，主要见于躯干和近端肢体，通常被视为低级别或“交界性”肿瘤。隆突性皮肤纤维肉瘤的临床特征为局部侵袭性生长和高局部复发率，但远处转移和肿瘤相关死亡非常罕见。乳腺DFSP极为罕见。免疫组织化学方面，隆突性皮肤纤维肉瘤的特征是波形蛋白和CD34染色呈阳性。我们报告一例65岁女性患者，其巨大DFSP发生于数例经切除的纤维腺瘤和神经纤维瘤部位，行根治性乳房切除术和腋窝淋巴结清扫术治疗。围手术期的组织学和免疫组织化学图像显示了巨大非叶状肉瘤的特征。
