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Familial carcinoid tumors and subsequent cancers : a nation-wide epidemiologic study from Sweden

Hemminki, K LU and Li, Xinjun LU (2001) In International Journal of Cancer 94(3). p.8-444
Abstract

Carcinoids are rare neuroendocrine tumors, mainly located in the bowel, stomach and lung. Familial risks in carcinoid tumours are not well known apart from multiple endocrine neoplasia 1 (MEN1). We used the nation-wide Swedish Family-Cancer Database on 10.1 million individuals for assessment. Carcinoid tumors were retrieved from the Cancer Registry covering the years 1958-1998. The offspring generation, aged 0-66 years, accumulated 190 million person-years at risk. The age-adjusted incidence rates were 0.76 for men and 1.29/100,000 for women. Standardized incidence ratios (SIRs) were calculated for offspring when their parents had a carcinoid or any other cancer. When parents presented with carcinoids, SIRs for offspring were 4.35 (n =... (More)

Carcinoids are rare neuroendocrine tumors, mainly located in the bowel, stomach and lung. Familial risks in carcinoid tumours are not well known apart from multiple endocrine neoplasia 1 (MEN1). We used the nation-wide Swedish Family-Cancer Database on 10.1 million individuals for assessment. Carcinoid tumors were retrieved from the Cancer Registry covering the years 1958-1998. The offspring generation, aged 0-66 years, accumulated 190 million person-years at risk. The age-adjusted incidence rates were 0.76 for men and 1.29/100,000 for women. Standardized incidence ratios (SIRs) were calculated for offspring when their parents had a carcinoid or any other cancer. When parents presented with carcinoids, SIRs for offspring were 4.35 (n = 8, 95% CI 1.86-7.89) for small intestinal and 4.65 (n = 4, 95% CI 1.21-10.32) for colon carcinoids. If both offspring and parents presented with small intestinal carcinoids, the SIR was 12.31 (n = 4, 95% CI 3.20-27.34). Offspring carcinoids were also increased if parents presented with bladder and endocrine gland tumors, the latter association probably partially due to MEN1. Risks for second cancers were increased, particularly at sites where familial risks were found, including carcinoids in the small intestine.

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Contribution to journal
publication status
published
keywords
Adolescent, Adult, Age of Onset, Aged, Carcinoid Tumor/epidemiology, Child, Child, Preschool, Colonic Neoplasms/epidemiology, Databases as Topic, Endocrine Gland Neoplasms/epidemiology, Family Health, Female, Genetic Predisposition to Disease, Humans, Infant, Infant, Newborn, Intestinal Neoplasms/epidemiology, Male, Middle Aged, Neoplasm Recurrence, Local/epidemiology, Neoplasms/epidemiology, Neoplasms, Second Primary/epidemiology, Recurrence, Risk Factors, Sex Factors, Sweden, Time Factors, Urinary Bladder Neoplasms/epidemiology
in
International Journal of Cancer
volume
94
issue
3
pages
8 - 444
publisher
John Wiley & Sons Inc.
external identifiers
  • pmid:11745428
  • scopus:0035501567
ISSN
0020-7136
DOI
10.1002/ijc.1473
language
English
LU publication?
no
id
e032262f-d4ad-400e-90cc-64eb03eb7e17
date added to LUP
2019-01-30 12:12:38
date last changed
2024-04-01 20:46:55
@article{e032262f-d4ad-400e-90cc-64eb03eb7e17,
  abstract     = {{<p>Carcinoids are rare neuroendocrine tumors, mainly located in the bowel, stomach and lung. Familial risks in carcinoid tumours are not well known apart from multiple endocrine neoplasia 1 (MEN1). We used the nation-wide Swedish Family-Cancer Database on 10.1 million individuals for assessment. Carcinoid tumors were retrieved from the Cancer Registry covering the years 1958-1998. The offspring generation, aged 0-66 years, accumulated 190 million person-years at risk. The age-adjusted incidence rates were 0.76 for men and 1.29/100,000 for women. Standardized incidence ratios (SIRs) were calculated for offspring when their parents had a carcinoid or any other cancer. When parents presented with carcinoids, SIRs for offspring were 4.35 (n = 8, 95% CI 1.86-7.89) for small intestinal and 4.65 (n = 4, 95% CI 1.21-10.32) for colon carcinoids. If both offspring and parents presented with small intestinal carcinoids, the SIR was 12.31 (n = 4, 95% CI 3.20-27.34). Offspring carcinoids were also increased if parents presented with bladder and endocrine gland tumors, the latter association probably partially due to MEN1. Risks for second cancers were increased, particularly at sites where familial risks were found, including carcinoids in the small intestine.</p>}},
  author       = {{Hemminki, K and Li, Xinjun}},
  issn         = {{0020-7136}},
  keywords     = {{Adolescent; Adult; Age of Onset; Aged; Carcinoid Tumor/epidemiology; Child; Child, Preschool; Colonic Neoplasms/epidemiology; Databases as Topic; Endocrine Gland Neoplasms/epidemiology; Family Health; Female; Genetic Predisposition to Disease; Humans; Infant; Infant, Newborn; Intestinal Neoplasms/epidemiology; Male; Middle Aged; Neoplasm Recurrence, Local/epidemiology; Neoplasms/epidemiology; Neoplasms, Second Primary/epidemiology; Recurrence; Risk Factors; Sex Factors; Sweden; Time Factors; Urinary Bladder Neoplasms/epidemiology}},
  language     = {{eng}},
  month        = {{11}},
  number       = {{3}},
  pages        = {{8--444}},
  publisher    = {{John Wiley & Sons Inc.}},
  series       = {{International Journal of Cancer}},
  title        = {{Familial carcinoid tumors and subsequent cancers : a nation-wide epidemiologic study from Sweden}},
  url          = {{http://dx.doi.org/10.1002/ijc.1473}},
  doi          = {{10.1002/ijc.1473}},
  volume       = {{94}},
  year         = {{2001}},
}