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

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

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)	951-5
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 - Oct 2007
Externally published	Yes

Keywords

Adult
Aged
Aged, 80 and over
Breast Neoplasms/diagnosis
Colorectal Neoplasms/diagnosis
Digestive System Neoplasms/epidemiology
Female
Humans
Incidence
Male
Middle Aged
Neoplasms, Multiple Primary/diagnosis
Netherlands/epidemiology
Paget Disease, Extramammary/epidemiology
Prognosis
Prostatic Neoplasms/diagnosis
Risk Factors
Skin Neoplasms/epidemiology
Survival Analysis

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10.1016/j.ejso.2006.11.028

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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 = "Adult, Aged, Aged, 80 and over, Breast Neoplasms/diagnosis, Colorectal Neoplasms/diagnosis, Digestive System Neoplasms/epidemiology, Female, Humans, Incidence, Male, Middle Aged, Neoplasms, Multiple Primary/diagnosis, Netherlands/epidemiology, Paget Disease, Extramammary/epidemiology, Prognosis, Prostatic Neoplasms/diagnosis, Risk Factors, Skin Neoplasms/epidemiology, Survival Analysis",

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

year = "2007",

month = oct,

doi = "10.1016/j.ejso.2006.11.028",

language = "English",

volume = "33",

pages = "951--5",

journal = "European Journal of Surgical Oncology",

issn = "0748-7983",

publisher = "W.B. Saunders Ltd",

number = "8",

}

TY - JOUR

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

Y1 - 2007/10

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 - Adult

KW - Aged

KW - Aged, 80 and over

KW - Breast Neoplasms/diagnosis

KW - Colorectal Neoplasms/diagnosis

KW - Digestive System Neoplasms/epidemiology

KW - Female

KW - Humans

KW - Incidence

KW - Male

KW - Middle Aged

KW - Neoplasms, Multiple Primary/diagnosis

KW - Netherlands/epidemiology

KW - Paget Disease, Extramammary/epidemiology

KW - Prognosis

KW - Prostatic Neoplasms/diagnosis

KW - Risk Factors

KW - Skin Neoplasms/epidemiology

KW - Survival Analysis

U2 - 10.1016/j.ejso.2006.11.028

DO - 10.1016/j.ejso.2006.11.028

M3 - Article

C2 - 17215101

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

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