Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis

Zeinab Ghorbanoghli; Barbara A. J. Bastiaansen; Alexandra M. J. Langers; Fokko M. Nagengast; Jan-Werner Poley; James C. H. Hardwick; Jan J. Koornstra; Silvia Sanduleanu; Wouter H. de Vos tot Nederveen Cappel; Ben J. M. Witteman; H. Morreau; Evelien Dekker; Hans F. A. Vasen

doi:10.1136/jmedgenet-2017-104545

Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis

Zeinab Ghorbanoghli, Barbara A. J. Bastiaansen, Alexandra M. J. Langers, Fokko M. Nagengast, Jan-Werner Poley, James C. H. Hardwick, Jan J. Koornstra, Silvia Sanduleanu, Wouter H. de Vos tot Nederveen Cappel, Ben J. M. Witteman, H. Morreau, Evelien Dekker, Hans F. A. Vasen^*

^*Corresponding author for this work

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

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Abstract

Background: Screening of patients with familial adenomatous polyposis (FAP) have led to a substantial reduction in mortality due to colorectal cancer (CRC). Recent guidelines suggest that surveillance of nonintestinal malignancies should also be considered in those patients. However, the value of these surveillance programmes is unknown. The aims of this study were (1) to assess the occurrence of extracolonic malignancies in a large series of adenomatous polyposis coli (APC) mutation carriers and (2) to evaluate the causes of death.

Methods: All APC mutation carriers were selected from the Dutch polyposis registry. Data on causes of death were collected. Pathology reports were retrieved from the Dutch Pathology Registry.

Results: A total of 85 extracolonic malignancies were diagnosed in 74 of 582 APC mutation carriers. Duodenal and skin cancers were the most prevalent cancers. Thyroid cancer was observed in only 1.5% of the cases. The main cause of death was cancer (59% of all deaths), with 42% due to CRC and 21% due to duodenal cancer. One patient died from thyroid cancer. The second and third most common causes of death were cardiovascular disease (13% of all deaths) and desmoid tumours (11% of all deaths), respectively.

Conclusion: Extending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.

Original language	English
Pages (from-to)	11-14
Number of pages	4
Journal	Journal of Medical Genetics
Volume	55
Issue number	1
DOIs	https://doi.org/10.1136/jmedgenet-2017-104545
Publication status	Published - 1-Jan-2018

Keywords

FAMILIAL ADENOMATOUS POLYPOSIS
DUODENAL ADENOMATOSIS
REGISTRATION
NETHERLANDS
PREVALENCE
MANAGEMENT
CARCINOMA
GENE

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Extracolonic cancer risk in Dutch patients with APC-associated polyposisFinal publisher's version, 228 KBLicence: Taverne

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Ghorbanoghli, Z., Bastiaansen, B. A. J., Langers, A. M. J., Nagengast, F. M., Poley, J.-W., Hardwick, J. C. H., Koornstra, J. J., Sanduleanu, S., Cappel, W. H. D. V. T. N., Witteman, B. J. M., Morreau, H., Dekker, E., & Vasen, H. F. A. (2018). Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis. Journal of Medical Genetics, 55(1), 11-14. https://doi.org/10.1136/jmedgenet-2017-104545

@article{1f622b129f3a4b528720da3f8b72a7f0,

title = "Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis",

abstract = "Background: Screening of patients with familial adenomatous polyposis (FAP) have led to a substantial reduction in mortality due to colorectal cancer (CRC). Recent guidelines suggest that surveillance of nonintestinal malignancies should also be considered in those patients. However, the value of these surveillance programmes is unknown. The aims of this study were (1) to assess the occurrence of extracolonic malignancies in a large series of adenomatous polyposis coli (APC) mutation carriers and (2) to evaluate the causes of death.Methods: All APC mutation carriers were selected from the Dutch polyposis registry. Data on causes of death were collected. Pathology reports were retrieved from the Dutch Pathology Registry.Results: A total of 85 extracolonic malignancies were diagnosed in 74 of 582 APC mutation carriers. Duodenal and skin cancers were the most prevalent cancers. Thyroid cancer was observed in only 1.5% of the cases. The main cause of death was cancer (59% of all deaths), with 42% due to CRC and 21% due to duodenal cancer. One patient died from thyroid cancer. The second and third most common causes of death were cardiovascular disease (13% of all deaths) and desmoid tumours (11% of all deaths), respectively.Conclusion: Extending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.",

keywords = "FAMILIAL ADENOMATOUS POLYPOSIS, DUODENAL ADENOMATOSIS, REGISTRATION, NETHERLANDS, PREVALENCE, MANAGEMENT, CARCINOMA, GENE",

author = "Zeinab Ghorbanoghli and Bastiaansen, {Barbara A. J.} and Langers, {Alexandra M. J.} and Nagengast, {Fokko M.} and Jan-Werner Poley and Hardwick, {James C. H.} and Koornstra, {Jan J.} and Silvia Sanduleanu and Cappel, {Wouter H. de Vos tot Nederveen} and Witteman, {Ben J. M.} and H. Morreau and Evelien Dekker and Vasen, {Hans F. A.}",

year = "2018",

month = jan,

day = "1",

doi = "10.1136/jmedgenet-2017-104545",

language = "English",

volume = "55",

pages = "11--14",

journal = "Journal of Medical Genetics",

issn = "0022-2593",

publisher = "BMJ Publishing Group",

number = "1",

}

Ghorbanoghli, Z, Bastiaansen, BAJ, Langers, AMJ, Nagengast, FM, Poley, J-W, Hardwick, JCH, Koornstra, JJ, Sanduleanu, S, Cappel, WHDVTN, Witteman, BJM, Morreau, H, Dekker, E & Vasen, HFA 2018, 'Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis', Journal of Medical Genetics, vol. 55, no. 1, pp. 11-14. https://doi.org/10.1136/jmedgenet-2017-104545

TY - JOUR

T1 - Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis

AU - Ghorbanoghli, Zeinab

AU - Bastiaansen, Barbara A. J.

AU - Langers, Alexandra M. J.

AU - Nagengast, Fokko M.

AU - Poley, Jan-Werner

AU - Hardwick, James C. H.

AU - Koornstra, Jan J.

AU - Sanduleanu, Silvia

AU - Cappel, Wouter H. de Vos tot Nederveen

AU - Witteman, Ben J. M.

AU - Morreau, H.

AU - Dekker, Evelien

AU - Vasen, Hans F. A.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Background: Screening of patients with familial adenomatous polyposis (FAP) have led to a substantial reduction in mortality due to colorectal cancer (CRC). Recent guidelines suggest that surveillance of nonintestinal malignancies should also be considered in those patients. However, the value of these surveillance programmes is unknown. The aims of this study were (1) to assess the occurrence of extracolonic malignancies in a large series of adenomatous polyposis coli (APC) mutation carriers and (2) to evaluate the causes of death.Methods: All APC mutation carriers were selected from the Dutch polyposis registry. Data on causes of death were collected. Pathology reports were retrieved from the Dutch Pathology Registry.Results: A total of 85 extracolonic malignancies were diagnosed in 74 of 582 APC mutation carriers. Duodenal and skin cancers were the most prevalent cancers. Thyroid cancer was observed in only 1.5% of the cases. The main cause of death was cancer (59% of all deaths), with 42% due to CRC and 21% due to duodenal cancer. One patient died from thyroid cancer. The second and third most common causes of death were cardiovascular disease (13% of all deaths) and desmoid tumours (11% of all deaths), respectively.Conclusion: Extending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.

AB - Background: Screening of patients with familial adenomatous polyposis (FAP) have led to a substantial reduction in mortality due to colorectal cancer (CRC). Recent guidelines suggest that surveillance of nonintestinal malignancies should also be considered in those patients. However, the value of these surveillance programmes is unknown. The aims of this study were (1) to assess the occurrence of extracolonic malignancies in a large series of adenomatous polyposis coli (APC) mutation carriers and (2) to evaluate the causes of death.Methods: All APC mutation carriers were selected from the Dutch polyposis registry. Data on causes of death were collected. Pathology reports were retrieved from the Dutch Pathology Registry.Results: A total of 85 extracolonic malignancies were diagnosed in 74 of 582 APC mutation carriers. Duodenal and skin cancers were the most prevalent cancers. Thyroid cancer was observed in only 1.5% of the cases. The main cause of death was cancer (59% of all deaths), with 42% due to CRC and 21% due to duodenal cancer. One patient died from thyroid cancer. The second and third most common causes of death were cardiovascular disease (13% of all deaths) and desmoid tumours (11% of all deaths), respectively.Conclusion: Extending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.

KW - FAMILIAL ADENOMATOUS POLYPOSIS

KW - DUODENAL ADENOMATOSIS

KW - REGISTRATION

KW - NETHERLANDS

KW - PREVALENCE

KW - MANAGEMENT

KW - CARCINOMA

KW - GENE

U2 - 10.1136/jmedgenet-2017-104545

DO - 10.1136/jmedgenet-2017-104545

M3 - Article

SN - 0022-2593

VL - 55

SP - 11

EP - 14

JO - Journal of Medical Genetics

JF - Journal of Medical Genetics

IS - 1

ER -

Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis

Abstract

Keywords

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