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Predominance of girls with cancer in families with multiple childhood cancer cases

Stjernfelt, Karl Johan LU ; von Stedingk, Kristoffer LU ; Wiebe, Thomas LU ; Hjorth, Lars LU ; Olsson, Håkan LU and Øra, Ingrid LU (2017) In BMC Cancer 17(1).
Abstract

Background: Recent studies indicate that one of four childhood cancers can be attributed to hereditary genetic abnormalities. Methods: The Lund Childhood Cancer Genetic study includes newly diagnosed childhood cancer patients as well as childhood cancer survivors visiting the Department of Pediatrics or the Late Effect Clinic at Skåne University Hospital, Lund, Sweden. Questionnaires regarding family history of cancer and blood samples were provided. Reported data were validated and extended by use of the Swedish Population- and Cancer Registries. Demographics in families with one case of childhood cancer (FAM1) were investigated and compared to families with multiple cases of childhood cancer (FAM > 1) as well as to childhood cancer... (More)

Background: Recent studies indicate that one of four childhood cancers can be attributed to hereditary genetic abnormalities. Methods: The Lund Childhood Cancer Genetic study includes newly diagnosed childhood cancer patients as well as childhood cancer survivors visiting the Department of Pediatrics or the Late Effect Clinic at Skåne University Hospital, Lund, Sweden. Questionnaires regarding family history of cancer and blood samples were provided. Reported data were validated and extended by use of the Swedish Population- and Cancer Registries. Demographics in families with one case of childhood cancer (FAM1) were investigated and compared to families with multiple cases of childhood cancer (FAM > 1) as well as to childhood cancer in the general population. Results: Forty-one out of 528 families (7.8%) had more than one case of childhood cancer. In 23 families the affected children were relatives up to a 3rd degree (4.4%). In FAM > 1, 69.2% of the children with leukemia and 60% of those with tumors in the central nervous system (CNS) had a childhood relative with matching diagnosis, both significantly higher than expected. Significantly more female than male patients were observed in FAM > 1 compared to FAM1. This female predominance was most striking in childhood leukemia (77% female) and also, yet to a lesser extent, in CNS tumors (68% female). Conclusions: We conclude that the high proportion of children with leukemia or CNS tumors in FAM > 1 having a childhood relative with the same diagnosis suggests a hereditary background. Moreover, we report a female predominance in childhood leukemia and childhood CNS tumors in FAM > 1, which may indicate a hereditary gender-specific risk factor in these families.

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organization
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type
Contribution to journal
publication status
published
subject
keywords
Familial cancer predisposition, Genetic cancer susceptibility, Hereditary cancer syndrome, Pediatric cancer
in
BMC Cancer
volume
17
issue
1
publisher
BioMed Central
external identifiers
  • scopus:85038394967
  • wos:000418718900003
ISSN
1471-2407
DOI
10.1186/s12885-017-3899-8
language
English
LU publication?
yes
id
1a96730a-8a28-45e6-94af-ea85191e12d5
date added to LUP
2018-01-03 12:08:41
date last changed
2018-01-16 13:28:50
@article{1a96730a-8a28-45e6-94af-ea85191e12d5,
  abstract     = {<p>Background: Recent studies indicate that one of four childhood cancers can be attributed to hereditary genetic abnormalities. Methods: The Lund Childhood Cancer Genetic study includes newly diagnosed childhood cancer patients as well as childhood cancer survivors visiting the Department of Pediatrics or the Late Effect Clinic at Skåne University Hospital, Lund, Sweden. Questionnaires regarding family history of cancer and blood samples were provided. Reported data were validated and extended by use of the Swedish Population- and Cancer Registries. Demographics in families with one case of childhood cancer (FAM1) were investigated and compared to families with multiple cases of childhood cancer (FAM &gt; 1) as well as to childhood cancer in the general population. Results: Forty-one out of 528 families (7.8%) had more than one case of childhood cancer. In 23 families the affected children were relatives up to a 3rd degree (4.4%). In FAM &gt; 1, 69.2% of the children with leukemia and 60% of those with tumors in the central nervous system (CNS) had a childhood relative with matching diagnosis, both significantly higher than expected. Significantly more female than male patients were observed in FAM &gt; 1 compared to FAM1. This female predominance was most striking in childhood leukemia (77% female) and also, yet to a lesser extent, in CNS tumors (68% female). Conclusions: We conclude that the high proportion of children with leukemia or CNS tumors in FAM &gt; 1 having a childhood relative with the same diagnosis suggests a hereditary background. Moreover, we report a female predominance in childhood leukemia and childhood CNS tumors in FAM &gt; 1, which may indicate a hereditary gender-specific risk factor in these families.</p>},
  articleno    = {868},
  author       = {Stjernfelt, Karl Johan and von Stedingk, Kristoffer and Wiebe, Thomas and Hjorth, Lars and Olsson, Håkan and Øra, Ingrid},
  issn         = {1471-2407},
  keyword      = {Familial cancer predisposition,Genetic cancer susceptibility,Hereditary cancer syndrome,Pediatric cancer},
  language     = {eng},
  month        = {12},
  number       = {1},
  publisher    = {BioMed Central},
  series       = {BMC Cancer},
  title        = {Predominance of girls with cancer in families with multiple childhood cancer cases},
  url          = {http://dx.doi.org/10.1186/s12885-017-3899-8},
  volume       = {17},
  year         = {2017},
}