Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome?

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Standard

Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome? / Srikantharajah, Thanusha; Skovby, Flemming; Behrendt, Nille; Jemec, Gregor B.E.; Saunte, Ditte M.

I: Acta Dermatovenerologica Alpina, Pannonica et Adriatica, Bind 29, Nr. 3, 2020, s. 133-140.

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt

Harvard

Srikantharajah, T, Skovby, F, Behrendt, N, Jemec, GBE & Saunte, DM 2020, 'Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome?', Acta Dermatovenerologica Alpina, Pannonica et Adriatica, bind 29, nr. 3, s. 133-140. https://doi.org/10.15570/actaapa.2020.28

APA

Srikantharajah, T., Skovby, F., Behrendt, N., Jemec, G. B. E., & Saunte, D. M. (2020). Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome? Acta Dermatovenerologica Alpina, Pannonica et Adriatica, 29(3), 133-140. https://doi.org/10.15570/actaapa.2020.28

Vancouver

Srikantharajah T, Skovby F, Behrendt N, Jemec GBE, Saunte DM. Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome? Acta Dermatovenerologica Alpina, Pannonica et Adriatica. 2020;29(3):133-140. https://doi.org/10.15570/actaapa.2020.28

Author

Srikantharajah, Thanusha ; Skovby, Flemming ; Behrendt, Nille ; Jemec, Gregor B.E. ; Saunte, Ditte M. / Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome?. I: Acta Dermatovenerologica Alpina, Pannonica et Adriatica. 2020 ; Bind 29, Nr. 3. s. 133-140.

Bibtex

@article{73dea1aaa5c9400b9f75fab01e2905e0,
title = "Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome?",
abstract = "Brooke–Spiegler syndrome (BSS) is a rare inherited autosomal dominant disease characterized by the development of multiple adnexal cutaneous neoplasms. BSS has been linked to mutations in CYLD gene, which is a tumor suppressor gene located on chromosome 16q12-q13. An increased risk of malignant transformation of adnexal cutaneous tumors in BSS patients has been reported. However, no reported genetic markers identify patients at risk of cutaneous malignancy. This study reviews published cases of BSS to investigate the role of clinical parameters as biomarkers of skin malignancy. A comprehensive review of the clinical aspects of BSS is based on 55 case reports. Our analysis revealed only age as a predictor of malignancy; however, this is also a general risk factor for development of malignancy and therefore of limited value as a screening tool. The study highlights the need for standardized clinical follow-up of patients.",
keywords = "Brooke–Spiegler syndrome, Genetics, Malignancy, Predictors",
author = "Thanusha Srikantharajah and Flemming Skovby and Nille Behrendt and Jemec, {Gregor B.E.} and Saunte, {Ditte M.}",
year = "2020",
doi = "10.15570/actaapa.2020.28",
language = "English",
volume = "29",
pages = "133--140",
journal = "Acta Dermatovenerologica Alpina, Panonica et Adriatica",
issn = "1318-4458",
publisher = "Slovenian Medical Society",
number = "3",

}

RIS

TY - JOUR

T1 - Can we clinically identify patients at risk of malignant transformation of skin tumors in Brooke–Spiegler syndrome?

AU - Srikantharajah, Thanusha

AU - Skovby, Flemming

AU - Behrendt, Nille

AU - Jemec, Gregor B.E.

AU - Saunte, Ditte M.

PY - 2020

Y1 - 2020

N2 - Brooke–Spiegler syndrome (BSS) is a rare inherited autosomal dominant disease characterized by the development of multiple adnexal cutaneous neoplasms. BSS has been linked to mutations in CYLD gene, which is a tumor suppressor gene located on chromosome 16q12-q13. An increased risk of malignant transformation of adnexal cutaneous tumors in BSS patients has been reported. However, no reported genetic markers identify patients at risk of cutaneous malignancy. This study reviews published cases of BSS to investigate the role of clinical parameters as biomarkers of skin malignancy. A comprehensive review of the clinical aspects of BSS is based on 55 case reports. Our analysis revealed only age as a predictor of malignancy; however, this is also a general risk factor for development of malignancy and therefore of limited value as a screening tool. The study highlights the need for standardized clinical follow-up of patients.

AB - Brooke–Spiegler syndrome (BSS) is a rare inherited autosomal dominant disease characterized by the development of multiple adnexal cutaneous neoplasms. BSS has been linked to mutations in CYLD gene, which is a tumor suppressor gene located on chromosome 16q12-q13. An increased risk of malignant transformation of adnexal cutaneous tumors in BSS patients has been reported. However, no reported genetic markers identify patients at risk of cutaneous malignancy. This study reviews published cases of BSS to investigate the role of clinical parameters as biomarkers of skin malignancy. A comprehensive review of the clinical aspects of BSS is based on 55 case reports. Our analysis revealed only age as a predictor of malignancy; however, this is also a general risk factor for development of malignancy and therefore of limited value as a screening tool. The study highlights the need for standardized clinical follow-up of patients.

KW - Brooke–Spiegler syndrome

KW - Genetics

KW - Malignancy

KW - Predictors

U2 - 10.15570/actaapa.2020.28

DO - 10.15570/actaapa.2020.28

M3 - Journal article

C2 - 32975300

AN - SCOPUS:85091616074

VL - 29

SP - 133

EP - 140

JO - Acta Dermatovenerologica Alpina, Panonica et Adriatica

JF - Acta Dermatovenerologica Alpina, Panonica et Adriatica

SN - 1318-4458

IS - 3

ER -

ID: 251358196