Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
Ann Surg Oncol. 2011 Feb;18(2):328-36. doi: 10.1245/s10434-010-1316-5. Epub 2010 Sep 16.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue malignancy that typically presents with local invasion but rarely metastasizes. We examine clinicopathologic factors associated with disease-free survival (DFS) in patients with primary and recurrent DFSP and evaluate responses to multimodality therapy.
Patients treated for DFSP were identified in a prospectively maintained database. Clinicopathologic factors associated with DFS were analyzed using univariate and multivariate analysis.
A total of 244 patients with DFSP were identified. Median follow-up was 50 months. A total of 14 patients had local recurrence (LR), and 2 patients had distant recurrence (DR), with a median time to recurrence of 35 months. At time of last follow-up, 70% and 47% of patients showed no evidence of disease (NED) in the primary (n = 197) and recurrent groups (n = 47), respectively. On univariate analysis, tumor location and depth were associated with DFS in the primary group, while margin status (R1 vs. R0) was associated with DFS in the LR group. On multivariate analysis, only depth (primary group) and margin status (LR group), remained significant. Also, 22 patients had therapy other than surgical resection: 14 radiotherapy, 4 tyrosine kinase inhibitor (TKI) only, 2 conventional chemotherapy only, and 2 chemotherapy plus TKI. Responses to other therapies were variable.
DFS after treatment for DFSP is strongly predicted by tumor depth in the primary setting and margin status in recurrent tumors. The treatment for DFSP in the primary or recurrent setting is excision with negative margins, resulting in low recurrence rates and infrequent metastatic spread. Multimodality treatment, especially TKI use, can be effective, but is not curative.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的软组织恶性肿瘤，通常表现为局部侵袭，但很少转移。我们研究了与原发性和复发性 DFSP 患者无病生存（DFS）相关的临床病理因素，并评估了多模式治疗的反应。
在一个前瞻性维护的数据库中确定了接受 DFSP 治疗的患者。使用单变量和多变量分析来分析与 DFS 相关的临床病理因素。
共确定了 244 例 DFSP 患者。中位随访时间为 50 个月。共有 14 例患者出现局部复发（LR），2 例患者出现远处复发（DR），复发时间中位数为 35 个月。在最后一次随访时，原发性（n = 197）和复发性组（n = 47）中分别有 70%和 47%的患者无疾病证据（NED）。单变量分析显示，肿瘤位置和深度与原发性组的 DFS 相关，而边缘状态（R1 与 R0）与 LR 组的 DFS 相关。多变量分析显示，只有深度（原发性组）和边缘状态（LR 组）仍然具有显著意义。此外，22 例患者接受了手术切除以外的治疗：14 例接受放疗，4 例仅接受酪氨酸激酶抑制剂（TKI），2 例仅接受常规化疗，2 例接受化疗加 TKI。其他治疗的反应各不相同。
原发性 DFSP 治疗后的 DFS 强烈预测肿瘤深度，复发性肿瘤的 DFS 强烈预测边缘状态。原发性或复发性 DFSP 的治疗是切除并保持阴性边缘，从而导致低复发率和罕见的转移性扩散。多模式治疗，特别是 TKI 的使用，可以有效，但不能治愈。
