Kim Minhee, Li Minmin, Intong-Wheeler Lizbeth R A, Tran Kim, Marucci Damian, Murrell Dedee F
Department of Dermatology, George Hospital, Gray St, Kogarah, Sydney, Australia.
Acta Derm Venereol. 2018 Jan 12;98(1):70-76. doi: 10.2340/00015555-2781.
We investigate the epidemiology and outcomes of squamous cell carcinoma (SCC) in recessive dystrophic epidermolysis bullosa (RDEB) from the Australasian EB registry cohort. Seventeen out of 49 (34.6%) RDEB patients developed at least one SCC. Data detailing SCC was obtainable from 16/17 RDEB-SCC patients. A total number of 161 primary SCCs occurred in 16 RDEB-SCC patients with a mean of 10 SCCs per person. The earliest age of first SCC development was 16 years. Eleven out of 16 RDEB-SCC patients eventually developed metastatic SCCs. The majority of the tumours were well and moderately differentiated. The cumulative risk of SCC development by age 35 was 76.1% for RDEB-Generalized Severe (RDEB-GS) and 10% for RDEB-Generalized Intermediate (RDEB-GI). Amongst those who developed SCCs, their median time to death after first SCC was 5 years for RDEB-GI and 4 years for RDEB-GS. This is the first retrospective study of RDEB-SCC in Australasia.
我们通过澳大利亚大表皮松解性大疱病（EB）登记队列，研究隐性营养不良性大疱性表皮松解症（RDEB）中鳞状细胞癌（SCC）的流行病学及预后情况。49例RDEB患者中有17例（34.6%）发生了至少1例SCC。16/17例RDEB-SCC患者有详细的SCC数据。16例RDEB-SCC患者共发生161例原发性SCC，平均每人10例。首次发生SCC的最早年龄为16岁。16例RDEB-SCC患者中有11例最终发生了转移性SCC。大多数肿瘤为高分化和中分化。35岁时RDEB-全身重度型（RDEB-GS）发生SCC的累积风险为76.1%，RDEB-全身中度型（RDEB-GI）为10%。在发生SCC的患者中，RDEB-GI患者首次发生SCC后至死亡的中位时间为5年，RDEB-GS患者为4年。这是澳大利亚首例关于RDEB-SCC的回顾性研究。
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