Department of Surgery and Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute of Jeonbuk National University Hospital, Jeonbuk National University Medical School, 20 Geonji-ro, Deokjin-gu, Jeonju City, Jeollabuk-Do 54907, Republic of Korea.
Department of Radiology and Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute of Jeonbuk National University Hospital, Jeonbuk National University Medical School, 20 Geonji-ro, Deokjin-gu, Jeonju City, Jeollabuk-Do 54907, Republic of Korea.
Curr Med Imaging. 2024;20:e15734056309290. doi: 10.2174/0115734056309290240513101648.
Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue sarcoma, accounting for approximately 1% of all tumors; however, DFSP of the breast is extremely rare. Moreover, DFSP generally has a low malignant potential and is characterized by a high rate of local recurrence along with a small but definite risk of metastasis. The risk of metastasis is higher in fibrosarcomatous transformation in DFSP than in ordinary DFSP.
We have, herein, reported a case of a 61-year-old male patient with fibrosarcomatous transformation in DFSP. Preoperative Dynamic Contrastenhanced Magnetic Resonance Imaging (DCE-MRI) of the breast revealed an oval-shaped mass with heterogeneous internal enhancement, a large vessel embedded within, and a washout curve pattern on kinetic curve analysis. The mass exhibited a hyperintense signal on Diffusion-weighted Imaging (DWI), with a low apparent diffusion coefficient value. Histologically, the bland spindle tumor cells were arranged in a storiform pattern. Areas with the highest histological grade demonstrated increased cellularity, cytological atypia, and mitotic activity. Immunohistochemically, Ki-67 and p53 were highly expressed.
Recognizing the risk and accurately diagnosing fibrosarcomatous transformation in male breast DFSP are critical for improving prognosis and establishing appropriate treatment and follow-up plans. This emphasizes the significance of combining immunohistopathological features with DCE-MRI and DWI to assist clinicians in the early and accurate diagnosis of sarcomas arising from male breast DFSP.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的软组织肉瘤，约占所有肿瘤的 1%；然而，乳腺 DFSP 极为罕见。此外，DFSP 通常恶性程度较低，其特征为局部复发率高，且有较小但明确的转移风险。DFSP 中的纤维肉瘤样转化比普通 DFSP 具有更高的转移风险。
我们报告了 1 例 61 岁男性纤维肉瘤样转化的 DFSP 患者。术前乳腺动态对比增强磁共振成像（DCE-MRI）显示椭圆形肿块，内部不均匀强化，内部嵌入大血管，动力学曲线分析呈廓清曲线模式。肿块在弥散加权成像（DWI）上呈高信号，表观弥散系数值较低。组织学上，温和的梭形肿瘤细胞呈席纹状排列。组织学分级最高的区域表现为细胞增多、细胞异型性和有丝分裂活性增加。免疫组化显示 Ki-67 和 p53 高表达。
认识到男性乳腺 DFSP 中纤维肉瘤样转化的风险并准确诊断对于改善预后和制定适当的治疗和随访计划至关重要。这强调了将免疫组织病理学特征与 DCE-MRI 和 DWI 相结合，以帮助临床医生早期准确诊断源自男性乳腺 DFSP 的肉瘤的重要性。
