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Risk of solid tumors and hematological malignancy in persons with Turner and Klinefelter syndromes : A national cohort study

Ji, Jianguang LU ; Zöller, Bengt LU ; Sundquist, Jan LU and Sundquist, Kristina LU (2016) In International Journal of Cancer 139(4). p.754-758
Abstract

The risk of solid and hematological malignancy in patients with Turner syndrome, characterized by X chromosome monosomy in women, and Klinefelter syndrome, characterized with two and more X chromosomes in men, is not well established, but such evidence may have etiological implications on cancer development. We identified a total of 1,409 women with Turner syndrome and 1,085 men with Klinefelter syndrome from the Swedish Hospital Discharge and Outpatient Register. These individuals were further linked to the Swedish Cancer Register to examine the standardized incidence ratios (SIRs) of cancer using the general population without Turner and Klinefelter syndromes as reference. The overall risk of cancer was 1.34 for women with Turner... (More)

The risk of solid and hematological malignancy in patients with Turner syndrome, characterized by X chromosome monosomy in women, and Klinefelter syndrome, characterized with two and more X chromosomes in men, is not well established, but such evidence may have etiological implications on cancer development. We identified a total of 1,409 women with Turner syndrome and 1,085 men with Klinefelter syndrome from the Swedish Hospital Discharge and Outpatient Register. These individuals were further linked to the Swedish Cancer Register to examine the standardized incidence ratios (SIRs) of cancer using the general population without Turner and Klinefelter syndromes as reference. The overall risk of cancer was 1.34 for women with Turner syndrome; it was increased only for solid tumors. For a specific type of tumor, the risk of melanoma and central nervous system tumor was significantly increased. For persons with Klinefelter syndrome, the risk of solid tumors was decreased (SIR = 0.66), whereas the risk of hematological malignancy was increased (SIR = 2.72). Non-Hodgkin lymphoma and leukemia showed an increased SIR of 3.02 and 3.62, respectively. Our study supported the hypothesis that X chromosome plays an important role in the etiology of solid tumors. The underlying mechanisms for the increased incidence of non-Hodgkin lymphoma and leukemia in persons with Klinefelter syndrome need to be investigated further.

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author
organization
publishing date
type
Contribution to journal
publication status
epub
subject
keywords
hematological malignancy, Klinefelter syndrome, national databases, solid tumors, Turner syndrome
in
International Journal of Cancer
volume
139
issue
4
pages
5 pages
publisher
John Wiley & Sons
external identifiers
  • Scopus:84976598907
ISSN
0020-7136
DOI
10.1002/ijc.30126
language
English
LU publication?
yes
id
ec19b643-e36a-46e4-a8e5-bd9c6dfa256d
date added to LUP
2016-07-18 13:56:10
date last changed
2016-11-16 11:08:04
@misc{ec19b643-e36a-46e4-a8e5-bd9c6dfa256d,
  abstract     = {<p>The risk of solid and hematological malignancy in patients with Turner syndrome, characterized by X chromosome monosomy in women, and Klinefelter syndrome, characterized with two and more X chromosomes in men, is not well established, but such evidence may have etiological implications on cancer development. We identified a total of 1,409 women with Turner syndrome and 1,085 men with Klinefelter syndrome from the Swedish Hospital Discharge and Outpatient Register. These individuals were further linked to the Swedish Cancer Register to examine the standardized incidence ratios (SIRs) of cancer using the general population without Turner and Klinefelter syndromes as reference. The overall risk of cancer was 1.34 for women with Turner syndrome; it was increased only for solid tumors. For a specific type of tumor, the risk of melanoma and central nervous system tumor was significantly increased. For persons with Klinefelter syndrome, the risk of solid tumors was decreased (SIR = 0.66), whereas the risk of hematological malignancy was increased (SIR = 2.72). Non-Hodgkin lymphoma and leukemia showed an increased SIR of 3.02 and 3.62, respectively. Our study supported the hypothesis that X chromosome plays an important role in the etiology of solid tumors. The underlying mechanisms for the increased incidence of non-Hodgkin lymphoma and leukemia in persons with Klinefelter syndrome need to be investigated further.</p>},
  author       = {Ji, Jianguang and Zöller, Bengt and Sundquist, Jan and Sundquist, Kristina},
  issn         = {0020-7136},
  keyword      = {hematological malignancy,Klinefelter syndrome,national databases,solid tumors,Turner syndrome},
  language     = {eng},
  month        = {08},
  number       = {4},
  pages        = {754--758},
  publisher    = {ARRAY(0x81f4888)},
  series       = {International Journal of Cancer},
  title        = {Risk of solid tumors and hematological malignancy in persons with Turner and Klinefelter syndromes : A national cohort study},
  url          = {http://dx.doi.org/10.1002/ijc.30126},
  volume       = {139},
  year         = {2016},
}