Epidemiology and Outcome of Squamous Cell Carcinoma in Epidermolysis Bullosa in Australia and New Zealand

被引：38

作者：

Kim, Minhee ^{[1
,4
]}

Li, Minmin ^{[1
,4
]}

Intong-Wheeler, Lizbeth R. A. ^{[1
]}

Tran, Kim ^{[2
,4
]}

Marucci, Damian ^{[3
,4
]}

Murrell, Dedee F. ^{[1
,4
]}

机构：

[1] St George Hosp, Dept Dermatol, Gray St, Sydney, NSW 2217, Australia

[2] SEALS, Dept Anat Pathol, Sydney, NSW, Australia

[3] St George Hosp, Dept Plast Surg, Sydney, NSW, Australia

[4] Univ New South Wales, Sydney, NSW, Australia

来源：

ACTA DERMATO-VENEREOLOGICA | 2018年 / 98卷 / 01期

关键词：

recessive dystrophic epidermolysis bullosa; squamous cell carcinoma; epidemiology; prognosis; VII COLLAGEN; SKIN; MANAGEMENT; DIAGNOSIS; RISK;

D O I：

10.2340/00015555-2781

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

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 RDEBGS. This is the first retrospective study of RDEB-SCC in Australasia.

引用

页码：70 / 76

页数：7

共 34 条

[1] Involvement of p53 and p16 tumor suppressor genes in recessive dystrophic epidermolysis bullosa-associated squamous cell carcinoma [J].

Arbiser, JL ;

Fan, CY ;

Su, XB ;

van Emburgh, BO ;

Cerimele, F ;

Miller, MS ;

Harvell, J ;

Marinkovich, MP .

JOURNAL OF INVESTIGATIVE DERMATOLOGY, 2004, 123 (04) :788-790

[2] Basic fibroblast growth factor: A missing link between collagen VII, increased collagenase, and squamous cell carcinoma in recessive dystrophic epidermolysis bullosa [J].

Arbiser, JL ;

Fine, JD ;

Murrell, D ;

Paller, A ;

Connors, S ;

Keough, K ;

Marsh, E ;

Folkman, J .

MOLECULAR MEDICINE, 1998, 4 (03) :191-195

[3] Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification [J].

Fine, Jo-David ;

Bruckner-Tuderman, Leena ;

Eady, Robin A. J. ;

Bauer, Eugene A. ;

Bauer, Johann W. ;

Has, Cristina ;

Heagerty, Adrian ;

Hintner, Helmut ;

Hovnanian, Alain ;

Jonkman, Marcel F. ;

Leigh, Irene ;

Marinkovich, M. Peter ;

Martinez, Anna E. ;

McGrath, John A. ;

Mellerio, Jemima E. ;

Moss, Celia ;

Murrell, Dedee F. ;

Shimizu, Hiroshi ;

Uitto, Jouni ;

Woodley, David ;

Zambruno, Giovanna .

JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 2014, 70 (06) :1103-1126

[4] Epidermolysis bullosa and the risk of life-threatening cancers: The National EB Registry experience, 1986-2006 [J].

Fine, Jo-David ;

Johnson, Lorraine B. ;

Weiner, Madeline ;

Li, Kuo-Ping ;

Suchindran, Chirayath .

JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 2009, 60 (02) :203-211

[5] TISSUE STRESS AND TUMOR PROMOTION - POSSIBLE RELEVANCE TO EPIDERMOLYSIS BULLOSA [J].

GOLDBERG, GI ;

EISEN, AZ ;

BAUER, EA .

ARCHIVES OF DERMATOLOGY, 1988, 124 (05) :737-741

[6] Sunlight Effects on Immune System: Is There Something Else in addition to UV-Induced Immunosuppression? [J].

Gonzalez Maglio, D. H. ;

Paz, M. L. ;

Leoni, J. .

BIOMED RESEARCH INTERNATIONAL, 2016, 2016

[7] Innate sensing of microbial products promotes wound-induced skin cancer [J].

Hoste, Esther ;

Arwert, Esther N. ;

Lal, Rohit ;

South, Andrew P. ;

Salas-Alanis, Julio C. ;

Murrell, Dedee F. ;

Donati, Giacomo ;

Watt, Fiona M. .

NATURE COMMUNICATIONS, 2015, 6

[8] A novel homozygous splice site mutation in COL7A1 in a Chinese patient with severe recessive dystrophic epidermolysis bullosa and squamous cell carcinoma [J].

Huang, Lin ;

Wong, Yim-Ping ;

Burd, Andrew .

INTERNATIONAL JOURNAL OF DERMATOLOGY, 2011, 50 (01) :52-56

[9]

Kho YC, 2010, ARCH DERMATOL, V146, P635, DOI 10.1001/archdermatol.2010.109

[10] Use of cetuximab as an adjuvant agent to radiotherapy and surgery in recessive dystrophic epidermolysis bullosa with squamous cell carcinoma [J].

Kim, M. ;

Li, M. ;

Intong, L. R. A. ;

Tran, K. ;

Melbourne, W. ;

Marucci, D. ;

Bucci, J. ;

de Souza, P. ;

Mallesara, G. ;

Murrell, D. F. .

BRITISH JOURNAL OF DERMATOLOGY, 2013, 169 (01) :208-210

← 1 2 3 4 →