Epidemiology and treatment of extramammary Paget disease in the Netherlands

S. Siesling; M.A.G. Elferink; J.A.A.M. van Dijck; J.P.E.N. Pierie; W.A.M. Blokx

doi:10.1016/j.ejso.2006.11.028

Epidemiology and treatment of extramammary Paget disease in the Netherlands

S. Siesling^*
, M.A.G. Elferink
, J.A.A.M. van Dijck
, J.P.E.N. Pierie
, W.A.M. Blokx

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

110 Citations (Scopus)

24 Downloads (Pure)

Abstract

Aim: To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies.

Method: All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry.

Results: In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32% of patients with invasive EMPD and 35% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95% confidence interval 1.2-2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin.

Conclusions: For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.

Original language	English
Pages (from-to)	951-955
Number of pages	5
Journal	European journal of surgical oncology
Volume	33
Issue number	8
DOIs	https://doi.org/10.1016/j.ejso.2006.11.028
Publication status	Published - 1 Oct 2007
Externally published	Yes

Keywords

Extramammary cancer
Paget disease
Rare tumour
Risk of second cancer
Survival
n/a OA procedure

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1016/j.ejso.2006.11.028

Cite this

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title = "Epidemiology and treatment of extramammary Paget disease in the Netherlands",

abstract = "Aim: To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies.Method: All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry.Results: In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72\%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32\% of patients with invasive EMPD and 35\% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95\% confidence interval 1.2-2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin.Conclusions: For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.",

keywords = "Extramammary cancer, Paget disease, Rare tumour, Risk of second cancer, Survival, n/a OA procedure",

author = "S. Siesling and M.A.G. Elferink and \{van Dijck\}, J.A.A.M. and J.P.E.N. Pierie and W.A.M. Blokx",

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T1 - Epidemiology and treatment of extramammary Paget disease in the Netherlands

AU - Siesling, S.

AU - Elferink, M.A.G.

AU - van Dijck, J.A.A.M.

AU - Pierie, J.P.E.N.

AU - Blokx, W.A.M.

PY - 2007/10/1

Y1 - 2007/10/1

N2 - Aim: To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies.Method: All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry.Results: In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32% of patients with invasive EMPD and 35% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95% confidence interval 1.2-2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin.Conclusions: For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.

AB - Aim: To determine the incidence of EMPD and to describe its epidemiology, treatment, survival and the risk of developing other malignancies.Method: All cases of EMPD, diagnosed between 1989 and 2001, were selected from the Netherlands Cancer Registry.Results: In total, 178 cases of invasive and 48 cases of in situ EMPD had been registered. The overall relative 5-year survival for invasive tumours was 72%. Most patients with invasive as well as in situ cancer underwent surgery. Other malignancies were found in 32% of patients with invasive EMPD and 35% of patients with in situ EMPD. Patients had an increased risk of developing a second primary cancer (standardized incidence ratio: 1.7; 95% confidence interval 1.2-2.4). The most frequent localizations of the other cancers were the colorectum, the prostate, the breast and the extragenital skin.Conclusions: For EMPD, which is a rare disease in the Netherlands, there are no clear diagnostic and treatment guidelines. The prognosis is fairly good. A thorough search for other tumours is recommended for these patients.

KW - Extramammary cancer

KW - Paget disease

KW - Rare tumour

KW - Risk of second cancer

KW - Survival

KW - n/a OA procedure

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DO - 10.1016/j.ejso.2006.11.028

M3 - Article

C2 - 17215101

AN - SCOPUS:34548641733

SN - 0748-7983

VL - 33

SP - 951

EP - 955

JO - European journal of surgical oncology

JF - European journal of surgical oncology

IS - 8

ER -

Epidemiology and treatment of extramammary Paget disease in the Netherlands

Abstract

Keywords

UN SDGs

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