Kelly Joseph H, Christensen Linze, Frohm Marcus L
University of South Dakota Sanford School of Medicine.
S D Med. 2025 May;78(suppl 5):s30.
Dermatofibrosarcoma protuberans (DFSP) is an aggressive rare cutaneous tumor with a relatively high rate of local recurrence. Incidence in the United States has been reported around 4 occurrences per million people per year. Local recurrences have been reported between 2 to 19% of cases. These cases are at higher risk for sarcomatous transformation, increasing the risk for metastasis. Early and accurate diagnosis, coupled with appropriate management, is essential to minimize these risks. However, early on, DFSP is often misdiagnosed as a number of benign subcutaneous growths. Misdiagnosis often leads to incorrect or delayed treatment. This study extends previous research by evaluating whether access to dermatology care influences DFSP treatment outcomes in a larger cohort and larger geographical area.
This study is a retrospective review currently including 68 adults with histologically confirmed DFSP. The study evaluated three endpoints: rates of appropriate workup and diagnosis comparing dermatology and non-dermatology providers, rates of appropriate primary treatment between lesions with and without appropriate workup and diagnosis, and rates of positive margins or primary recurrence between lesions without and with appropriate primary treatment.
For all three endpoints, the data rejected the null hypothesis.
These findings further support that early biopsy and high clinical suspicion for DFSP improve surgical outcomes, particularly when diagnostic biopsy is performed prior to treatment. The larger sample size reinforces the importance of dermatology access in optimizing patient care and decreasing morbidity from DFSP treatment. Increased clinical awareness and stricter adherence to diagnostic and therapeutic guidelines are recommended for improved patient outcomes in the management of DFSP.
隆突性皮肤纤维肉瘤(DFSP)是一种侵袭性罕见皮肤肿瘤,局部复发率相对较高。据报道,美国每年每百万人中约有4例发病。局部复发率在2%至19%之间。这些病例发生肉瘤变的风险较高,转移风险增加。早期准确诊断并结合适当治疗对于将这些风险降至最低至关重要。然而,早期DFSP常被误诊为多种良性皮下肿物。误诊往往导致治疗不当或延误。本研究通过评估在更大队列和更大地理区域内获得皮肤科护理是否会影响DFSP的治疗结果,扩展了先前的研究。
本研究为回顾性研究,目前纳入了68例经组织学确诊的DFSP成年患者。该研究评估了三个终点:比较皮肤科和非皮肤科医生的适当检查和诊断率、有和没有适当检查和诊断的病变之间的适当初始治疗率,以及没有和有适当初始治疗的病变之间的切缘阳性或初次复发率。
对于所有三个终点,数据均拒绝了原假设。
这些发现进一步支持,早期活检和对DFSP的高度临床怀疑可改善手术结果,特别是在治疗前进行诊断性活检时。更大的样本量强化了获得皮肤科护理在优化患者护理和降低DFSP治疗发病率方面的重要性。建议提高临床意识并更严格地遵循诊断和治疗指南,以改善DFSP管理中的患者预后。