Safdar Fatima, Ud Din Nasir, Sattar Abida K, Fatima Syeda Samia, Idress Romana
Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, PAK.
Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, PAK.
Cureus. 2025 Jan 2;17(1):e76786. doi: 10.7759/cureus.76786. eCollection 2025 Jan.
Dermatofibrosarcoma protuberans (DFSP) of the breast is a rare entity. It is a slow-growing soft tissue tumor of low to intermediate grade. The risk of metastasis is low, but its likelihood of local recurrence is significant.
Our study aims to present the clinical and histological features of DFSP of breast and follow-up.
Patients with histologically proven DFSP between 01 January 2010 and 31 October 2023 were identified from a prospectively maintained pathology database. Two senior pathologists reviewed the clinical data and histological slides, and a follow-up was obtained.
Twenty-six cases of DFSP breast were diagnosed between 01 January 2010 and 31 October 2023. Out of 26, 10 (38.5%) were male and 16 (61.5%) were female. The mean age of presentation was 37.2 years in females and 40.7 years in males. The mean tumor size in females was 4.7 cm and in males was 5.4 cm. Histologically, the 15 DFSP cases (58%) showed spindle cells arranged in a storiform pattern with honeycomb-type fat infiltration. Fibrosarcomatous transformation was noted in 11 (42%) cases comprising a fascicular pattern. The median follow-up period was 6.1 years. Three (12%) patients experienced recurrence. No recurrence was observed in 23 (88%) patients with complete surgical resection.
We present the largest series of breast DFSP. The recurrence rate of 12% aligns with the DFSP of other common sites. Fibrosarcomatous transformation in breast DFSP (42%) is higher as compared to DFSP in other common locations and its long-term clinical behavior cannot be reliably predicted due to lack of long-term follow-up.
乳腺隆突性皮肤纤维肉瘤（DFSP）是一种罕见的疾病。它是一种生长缓慢的低至中级别的软组织肿瘤。转移风险较低，但其局部复发的可能性很大。
我们的研究旨在呈现乳腺DFSP的临床和组织学特征及随访情况。
从一个前瞻性维护的病理数据库中识别出2010年1月1日至2023年10月31日期间组织学确诊为DFSP的患者。两位资深病理学家审查了临床数据和组织学切片，并进行了随访。
2010年1月1日至2023年10月31日期间共诊断出26例乳腺DFSP。其中，10例（38.5%）为男性，16例（61.5%）为女性。女性的平均发病年龄为37.2岁，男性为40.7岁。女性的平均肿瘤大小为4.7厘米，男性为5.4厘米。组织学上，15例DFSP（58%）表现为呈车辐状排列的梭形细胞，伴有蜂窝状脂肪浸润。11例（42%）出现纤维肉瘤样转化，呈束状排列。中位随访期为6.1年。3例（12%）患者出现复发。23例（88%）接受完全手术切除的患者未观察到复发。
我们展示了最大系列的乳腺DFSP病例。12%的复发率与其他常见部位的DFSP一致。乳腺DFSP中的纤维肉瘤样转化（42%）高于其他常见部位的DFSP，由于缺乏长期随访，其长期临床行为无法可靠预测。
