J Drugs Dermatol. 2022 May 1;21(5):534-535. doi: 10.36849/JDD.6719.
Dermatofibrosarcoma protuberans (DFSP) is a rare, fibrohistiocytic tumor with intermediate malignancy.1 While these tumors are slow-growing and only metastasize in 6% of cases,2 they are often locally destructive, with relatively high local recurrence rates after initial excision. Overall annual incidence rates in the US are 0.8-4.1 per million person-years,2 though incidence among African Americans is nearly double that of Caucasians.3 DFSP is most commonly seen on the trunk (42-50%), followed by the extremities (30-42%) and, rarely, on the head and neck (10-15%).2,4 Other studies report that DFSP of the scalp accounts for under 5% of total cases.5 However, the head and neck region is reported to have the highest tendency to recur locally, roughly 50-75% of cases.4 Further, DFSP tumors on the scalp have the potential to metastases to the brain,4 thus highlighting the importance for these tumors to be correctly diagnosed and treated early on. Partly due to its rarity and also its tendency to mimic other mainly benign lesions clinically, DFSP is often misdiagnosed, leading to years of delay in proper treatment and otherwise likely avoidable sequelae.6 We describe a rare presentation of DFSP on the scalp of a 45-year-old African American woman successfully treated with "slow-Mohs" micrographic surgery. We also discuss the most common misdiagnoses for DFSP and scenarios when this tumor should be included in the differential and subsequent work-up.J Drugs Dermatol. 2022;21(5):534-535. doi:10.36849/JDD.6719.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的纤维组织细胞性肿瘤,具有中度恶性。1 尽管这些肿瘤生长缓慢,仅在 6%的病例中转移,2 但它们通常具有局部破坏性,初始切除后局部复发率相对较高。在美国,整体年发病率为每百万人每年 0.8-4.1 例,2 但非裔美国人的发病率几乎是白种人的两倍。3 DFSP 最常见于躯干(42-50%),其次是四肢(30-42%),很少见于头颈部(10-15%)。2,4 其他研究报告称,头皮 DFSP 占总病例的不到 5%。5 然而,头颈部被报道为局部复发倾向最高的部位,约 50-75%的病例。4 此外,头皮上的 DFSP 肿瘤有转移到大脑的潜力,4 因此强调了早期正确诊断和治疗这些肿瘤的重要性。部分由于其罕见性,以及其在临床上倾向于模仿其他主要良性病变的特点,DFSP 经常被误诊,导致多年来未能得到适当治疗,否则可能会出现可避免的后果。6 我们描述了一位 45 岁非裔美国女性头皮罕见的 DFSP 表现,成功地采用“慢-Mohs”显微手术进行了治疗。我们还讨论了 DFSP 最常见的误诊,并讨论了在哪些情况下应将该肿瘤纳入鉴别诊断和进一步检查。J 皮肤病药物杂志。2022;21(5):534-535. doi:10.36849/JDD.6719.