Felix Bryan, Kaza Suma
Medicine, Avalon University School of Medicine, Willemstad, CUW.
Pathology, Avalon University School of Medicine, Willemstad, CUW.
Cureus. 2024 Dec 12;16(12):e75591. doi: 10.7759/cureus.75591. eCollection 2024 Dec.
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive cutaneous sarcoma with a high propensity for recurrence, even following complete surgical excision. DFSP exhibits a low metastatic potential and is characterized by a distinctive honeycomb-like architecture composed of uniformly arranged spindle cells that frequently show CD34 immunostaining. Common surgical approaches include wide local excision (WLE), Mohs micrographic surgery (MMS), and, in severe cases, amputation. This literature review explores the impact of the surgical incision site on DFSP recurrence, placing particular emphasis on anatomically challenging regions, such as the head and neck, where achieving tumor-free margins is often difficult due to complex structures and limited tissue for resection. Our primary hypothesis is that DFSP cases arising in these intricate anatomical areas exhibit a higher recurrence risk compared to those on the trunk or extremities, where broader margins are more feasible. To investigate this hypothesis, data from a range of peer-reviewed studies and case reports were analyzed, including diverse patient populations from international sources, institutional case series, and large-scale database analyses, such as the Surveillance, Epidemiology, and End Results (SEER) Program. We evaluated recurrence rates, the adequacy of surgical margins, and anatomical influences across these studies while also focusing on histopathological findings like the presence of fibrosarcomatous (FS) variants, which are known to correlate with aggressive behavior and recurrence. We also reviewed emerging targeted therapies, particularly imatinib, as promising options for managing cases of unresectable or recurrent DFSP, thereby expanding therapeutic choices for clinicians when surgery alone proves inadequate. Our findings suggest a marked increase in recurrence risk for DFSP cases located in the head and neck region, attributed to limitations in achieving wide excision margins in these areas. This review underscores the importance of detailed preoperative planning, precise excision strategies, and individualized approaches based on tumor location to enhance surgical outcomes. Long-term surveillance remains crucial in DFSP management, particularly in high-risk locations, and continued research into targeted therapies offers hope for reducing recurrence rates and improving the quality of life for affected patients.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见的局部侵袭性皮肤肉瘤，即使在完全手术切除后仍有很高的复发倾向。DFSP转移潜能低，其特征是具有独特的蜂窝状结构，由排列均匀的梭形细胞组成，这些细胞常显示CD34免疫染色。常见的手术方法包括广泛局部切除（WLE）、莫氏显微外科手术（MMS），严重情况下需截肢。这篇文献综述探讨了手术切口部位对DFSP复发的影响，特别强调了解剖结构复杂的区域，如头颈部，由于结构复杂和可供切除的组织有限，在这些区域实现无瘤切缘往往很困难。我们的主要假设是，与躯干或四肢的DFSP病例相比，这些复杂解剖区域出现的DFSP病例复发风险更高，因为在躯干或四肢更容易获得更宽的切缘。为了验证这一假设，我们分析了一系列同行评审研究和病例报告的数据，包括来自国际来源的不同患者群体、机构病例系列以及大规模数据库分析，如监测、流行病学和最终结果（SEER）计划。我们评估了这些研究中的复发率、手术切缘的充分性以及解剖学影响，同时还关注组织病理学发现，如纤维肉瘤（FS）变体的存在，已知其与侵袭性生物学行为和复发相关。我们还回顾了新兴的靶向治疗方法，特别是伊马替尼，作为治疗不可切除或复发性DFSP病例的有前景的选择，从而在单独手术证明不足时为临床医生扩大治疗选择。我们的研究结果表明，位于头颈部区域的DFSP病例复发风险显著增加，这归因于在这些区域实现广泛切除切缘存在局限性。本综述强调了详细的术前规划、精确的切除策略以及基于肿瘤位置的个体化方法对提高手术效果的重要性。长期监测在DFSP的管理中仍然至关重要，特别是在高风险部位，并且对靶向治疗的持续研究为降低复发率和改善受影响患者的生活质量带来了希望。
