Zhou Bing-Jie, Zhong Hai-Yan, Chen Yong, Wang Qian, Wang Min, Zou Ming-Li, Yuan Si-Ming
Southeast University School of Medicine, Nanjing, Jiangsu, China.
Department of Plastic Surgery, Nanjing Jinling Hospital, Jinling Clinical Medical College, Nanjing University of Chinese Medicine, Nanjing, Jiangsu, China.
J Cosmet Dermatol. 2025 May;24(5):e70217. doi: 10.1111/jocd.70217.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy with aggressive growth.
To summarize the surgical treatment and results of DFSP based on a case series and literature review.
Seventeen DFSP cases were analyzed retrospectively at our hospital from June 2016 to December 2022, including 14 males and 3 females. All these cases were treated by wide local excision with negative margins. Postoperative follow-up was performed to observe recurrence, survival, and the effect of wound repair.
Routine postoperative pathology confirmed the morphological subtypes of these cases, including 12 cases of classical DFSP, 3 cases of DFSP-fibrosarcoma, 1 case of myxoid, and 1 case of Bednar. The median defect size was 72 cm (interquartile range 20-750 cm). All patients required split skin (47.1%) or local flap (52.9%) to reconstruct the soft tissue defect caused by tumor resection. All split-skin grafts were viable, and one flap case had marginal necrosis, which healed by dressing change. After 6-32 months of follow-up, local recurrence occurred in 1 case, and extensive intra-abdominal metastasis and death occurred in another case.
Wide local excision with negative margins can achieve a favorable prognosis in classical DFSP. The use of flaps for reconstruction results in better appearance and function.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的具有侵袭性生长的软组织恶性肿瘤。
基于病例系列和文献综述总结DFSP的手术治疗方法及结果。
回顾性分析2016年6月至2022年12月我院收治的17例DFSP病例,其中男性14例,女性3例。所有病例均采用切缘阴性的广泛局部切除治疗。术后进行随访,观察复发、生存及伤口修复效果。
术后常规病理确诊了这些病例的形态学亚型,包括12例经典型DFSP、3例DFSP-纤维肉瘤、1例黏液型和1例贝德纳型。缺损大小中位数为72 cm(四分位间距20 - 750 cm)。所有患者均需采用中厚皮片(47.1%)或局部皮瓣(52.9%)修复肿瘤切除所致的软组织缺损。所有中厚皮片移植均存活,1例皮瓣出现边缘坏死,经换药愈合。随访6 - 32个月,1例局部复发,另1例出现广泛腹腔内转移并死亡。
切缘阴性的广泛局部切除可使经典型DFSP获得良好预后。采用皮瓣修复可获得更好的外观和功能。