Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years' experience of a reference centre in Spain
Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years' experience of a reference centre in Spain
复制标题
DOI：
10.1007/s12094-019-02073-3
复制
发表时间：
2019-11-01
影响因子：
3.4
通讯作者：
de Lucas, R.
中科院分区：
文献类型：
--
作者：
Castelo, B.;Vinal, D.;de Lucas, R.
皮肤鳞状细胞癌(CSCC)是隐性营养不良性大疱性表皮松解症(RDEB)患者的主要死亡原因。我们回顾了1988年11月至2018年10月在拉巴斯大学医院就诊的RDEB患者。结果14例患者至少发生一例CSCC。肿瘤主要是分化良好的。近一半的肿瘤已经复发。首次复发的中位时间为23.4个月(95%CI：17.2~29.5)。有5名患者发生了远处转移。中位总生存期(MOS)为136.5个月(95%可信区间：30.6~242.3)。发生远处转移时的MOS为6.78个月(95%CI：1.94~11.61)。结论SCC是RDEB患者的一种危及生命的并发症。尽管肿瘤通常分化良好，但它们往往会复发。这是西班牙首例RDEB患者发生CSCC的报告。
BackgroundCutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center.Materials and methodsWe retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018.ResultsFourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2-29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6-242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94-11.61).ConclusionscSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.