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Title: Epidemiology and treatment of extramammary Paget disease in the Netherlands.
Author(s): Siesling, S.; Elferink, M.A.; Dijck, J.A.A.M. van ; Pierie, J.P.; Blokx, W.A.M.
Publication year: 2007
Source: European Journal of Surgical Oncology, vol. 33, iss. 8, (2007), pp. 951-955
ISSN: 0748-7983
DOI: https://doi.org/10.1016/j.ejso.2006.11.028
Publication type: Article / Letter to editor

Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/52016   https://hdl.handle.net/2066/52016

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Subject: CTR 3: Translational research
NCEBP 1: Molecular epidemiology
ONCOL 3: Translational research
UMCN 1.2: Molecular diagnosis, prognosis and monitoring
UMCN 1.5: Interventional oncology
UMCN 5.4: Renal disorders
Organization: Health Evidence
Pathology
Former Organization: Epidemiology, Biostatistics & HTA
Journal title:
European Journal of Surgical Oncology
Volume: vol. 33
Issue: iss. 8
Page start: p. 951
Page end: p. 955
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.

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