Pryor Elizabeth K, Sinkler Margaret A, Ullah Asad, Martin Elizabeth, Homlar Kelly
Orthopedics, Medical College of Georgia - Augusta University, Augusta, USA.
Pathology, Medical College of Georgia - Augusta University, Augusta, USA.
Cureus. 2021 Sep 18;13(9):e18079. doi: 10.7759/cureus.18079. eCollection 2021 Sep.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor originating from the dermis, with high rates of local recurrence and invasive growth but low likelihood of distant metastasis. Fibrosarcomatous transformation (FS-DFSP) of DFSP accounts for approximately 5-15% of DFSP tumors, is a higher-grade tumor, with higher chances of metastasis and poorer prognosis. We present a case of a 66-year-old female presented with a large fungating mass on the left dorsal foot. Ultrasound-guided core needle biopsy with immunohistochemistry suggested a spindle cell neoplasm, favoring myxofibrosarcoma with intermediate grade. The patient elected for below-knee amputation over limb salvage with wide resection and free flap reconstruction. Based on clinical presentation, radiologic, histologic features and fluorescence in situ hybridization (FISH) studies confirmed the diagnosis of fibrosarcomatous variant of dermatofibrosarcoma protuberans with myxoid change. FS-DFSP with myxoid change is a rare soft tissue tumor that requires aggressive treatment due to its high rates of recurrence. This case presents a rare tumor in a unique location that was successfully treated with limb amputation, which is not documented in current literature.
隆突性皮肤纤维肉瘤（DFSP）是一种起源于真皮的罕见软组织肿瘤，局部复发率和侵袭性生长率高，但远处转移的可能性低。DFSP的纤维肉瘤样转化（FS-DFSP）约占DFSP肿瘤的5%-15%，是一种高级别肿瘤，转移几率更高，预后更差。我们报告一例66岁女性，其左足背出现一个巨大的溃疡性肿块。超声引导下的粗针活检及免疫组化提示为梭形细胞瘤，倾向于中度黏液纤维肉瘤。患者选择膝下截肢而非保肢的广泛切除及游离皮瓣重建术。基于临床表现、影像学、组织学特征及荧光原位杂交（FISH）研究，确诊为伴有黏液样改变的隆突性皮肤纤维肉瘤纤维肉瘤样变体。伴有黏液样改变的FS-DFSP是一种罕见的软组织肿瘤，因其高复发率需要积极治疗。该病例展示了一个罕见肿瘤位于一个独特部位，通过肢体截肢成功治疗，目前文献中尚无记载。
