腹部和骨盆巨大达里埃-费朗隆突性皮肤纤维肉瘤：一例报告
Giant Darier-Ferrand dermatofibrosarcoma protuberans of the abdomen and pelvis: a case report.
作者信息
Triki A, Aloui M, Ghalleb M, Jbir I, Naceur Ben, Hassouna J Ben, Chargui R, Rahal K
机构信息
Surgical Oncology Department, Salah Azaiez Institute of Oncology, Tunis, Tunisia.
Faculty of Medicine, Tunis, Tunisia.
出版信息
J Med Case Rep. 2021 Mar 15;15(1):120. doi: 10.1186/s13256-021-02687-8.
BACKGROUND
Darier-Ferrand dermatofibrosarcoma (DFS) is a rare malignant cutaneous neoplasm characterized by local aggressiveness, high risk of recurrence, and low metastatic potential.
CASE PRESENTATION
A 60-year-old Tunisian man with recurrent abdominopelvic DFS for which he had undergone multiple excisions presented with an extensive DFS that invaded the external genitals. He underwent a large excision with emasculation and thin cutaneous graft of the abdominal wall and local skin flap in the pelvis.
CONCLUSION
DFS is a rare yet recurrent skin tumor. Wide excision with free margins remains the cornerstone of treatment. We report a case of a giant DFS treated with wide excision and reconstructive surgery to cover the defect.
背景
达里埃 - 费朗皮肤纤维肉瘤（DFS）是一种罕见的恶性皮肤肿瘤，其特点是局部侵袭性强、复发风险高且转移潜能低。
病例介绍
一名60岁的突尼斯男子，患有复发性腹盆腔DFS，已接受多次切除手术，此次出现广泛的DFS侵犯外生殖器。他接受了包括去势、腹壁薄皮片移植及盆腔局部皮瓣的大面积切除术。
结论
DFS是一种罕见但易复发的皮肤肿瘤。切缘阴性的广泛切除仍是治疗的基石。我们报告了一例通过广泛切除及重建手术修复缺损来治疗的巨大DFS病例。
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