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==Overview==
==Overview==


The [[prevalence]] of gonadoblastoma depends on the chromosomal content, presence or absence of [[mosaicism]], gonadal [[histology]], and age of the patient and varies between 15% to 30%. The [[incidence]] of gonadoblastoma varies according to the presence or absence of [[Y chromosome|Y chromosomal]] content and age of the individual. Patients with [[Turner syndrome]] who have [[Y chromosome|Y chromosomal]] content either completely or partially may develop gonadoblastoma with an incidence as high as 43%. Phenotypical females with XY [[gonadal]] abnormalities have been observed to develop gonadoblastoma by 40%. Gonadoblastoma may be found at any age, but commonly presents before the age of 15 years. Since, it usually affects individuals with [[gonadal dysgenesis]], there is no [[genotype]]-[[phenotype]] correlation. Phenotypically, it tends to affect female individuals to a greater extent.
The [[prevalence]] of gonadoblastoma depends on the chromosomal content, presence or absence of [[mosaicism]], gonadal [[histology]], and age of the patient and varies between 15,000 to 30,000 per 100,000 individuals worldwide. The [[incidence]] of gonadoblastoma varies according to the presence or absence of [[Y chromosome|Y chromosomal]] content and age of the individual. Patients with [[Turner syndrome]] who have [[Y chromosome|Y chromosomal]] content either completely or partially may develop gonadoblastoma with an incidence as high as 43,000 per 100,000 individuals worldwide. The incidence of gonadoblastoma among phenotypical females with XY [[gonadal]] abnormalities have been observed to be 40,000 per 100,000 individuals worldwide. Gonadoblastoma may be found at any age, but commonly presents before the age of 15 years. Since, it usually affects individuals with [[gonadal dysgenesis]], there is no [[genotype]]-[[phenotype]] correlation. Phenotypically, it tends to affect female individuals to a greater extent.




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===Incidence===
===Incidence===


*The [[incidence]] of gonadoblastoma varies according to the presence or absence of [[Y chromosome|Y chromosom]]<nowiki/>[[Y chromosome|al]] content and age of the individual.<ref name="pmid16600779">{{cite journal |vauthors=Brant WO, Rajimwale A, Lovell MA, Travers SH, Furness PD, Sorensen M, Oottamasathien S, Koyle MA |title=Gonadoblastoma and Turner syndrome |journal=J. Urol. |volume=175 |issue=5 |pages=1858–60 |date=May 2006 |pmid=16600779 |doi=10.1016/S0022-5347(05)0032-8 |url=}}</ref><ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref>
* The [[incidence]] of gonadoblastoma varies according to the presence or absence of [[Y chromosome|Y chromosom]]<nowiki/>[[Y chromosome|al]] content and age of the individual.<ref name="pmid16600779">{{cite journal |vauthors=Brant WO, Rajimwale A, Lovell MA, Travers SH, Furness PD, Sorensen M, Oottamasathien S, Koyle MA |title=Gonadoblastoma and Turner syndrome |journal=J. Urol. |volume=175 |issue=5 |pages=1858–60 |date=May 2006 |pmid=16600779 |doi=10.1016/S0022-5347(05)0032-8 |url=}}</ref><ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref>
*Phenotypical female with XY gonadal abnormalities have been observed to develop gonadoblastoma by 40%.
* The incidence of gonadoblastoma among phenotypical females with XY [[gonadal]] abnormalities have been observed to be 40,000 per 100,000 individuals worldwide.
*Patients with [[Turner syndrome]] who have [[Y chromosome|Y chromosom]]<nowiki/>[[Y chromosome|al]] content either completely or partially can develop gonadoblastoma with an [[incidence]] as high as 43%.
* Patients with [[Turner syndrome]] who have [[Y chromosome|Y chromosom]]<nowiki/>[[Y chromosome|al]] content either completely or partially can develop gonadoblastoma with an [[incidence]] as high as 43%.


===Prevalence===
===Prevalence===


*The [[prevalence]] of gonadoblastoma depends on the chromosomal content, presence or absence of [[mosaicism]], gonadal [[histology]] and age of the patient and varies between 15% to 30%.<ref name="urlYen & Jaffes Reproductive Endocrinology | ScienceDirect">{{cite web |url=https://www.sciencedirect.com/book/9781455727582/yen-and-jaffes-reproductive-endocrinology#book-info |title=Yen & Jaffe's Reproductive Endocrinology &#124; ScienceDirect |format= |work= |accessdate=}}</ref>
* The [[prevalence]] of gonadoblastoma depends on the chromosomal content, presence or absence of [[mosaicism]], gonadal [[histology]] and age of the patient and varies between 15,000 to 30,000 per 100,000 individuals worldwide.<ref name="urlYen & Jaffes Reproductive Endocrinology | ScienceDirect">{{cite web |url=https://www.sciencedirect.com/book/9781455727582/yen-and-jaffes-reproductive-endocrinology#book-info |title=Yen & Jaffe's Reproductive Endocrinology &#124; ScienceDirect |format= |work= |accessdate=}}</ref>


===Age===
===Age===


*Gonadoblastoma affects individuals of any age but commonly presents before 15 years of age.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }}</ref>
* Gonadoblastoma affects individuals of any age but commonly presents before 15 years of age.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }}</ref>


===Race===
===Race===


*There is no racial predilection to gonadoblastoma.
* There is no racial predilection to gonadoblastoma.


===Gender===
===Gender===


*It commonly originated from dysgenetic [[gonad]] but has been observed in women with normal [[ovary]] and [[karyotype]]<nowiki/>s, although rare.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }}</ref><ref name="pmid10226831">{{cite journal |vauthors=Hatano T, Yoshino Y, Kawashima Y, Shirai H, Iizuka N, Miyazawa Y, Sakata A, Onishi T |title=Case of gonadoblastoma in a 9-year-old boy without physical abnormalities |journal=Int. J. Urol. |volume=6 |issue=3 |pages=164–6 |date=March 1999 |pmid=10226831 |doi= |url=}}</ref>
* It commonly originated from dysgenetic [[gonad]] but has been observed in women with normal [[ovary]] and [[karyotype]]<nowiki/>s, although rare.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }}</ref><ref name="pmid10226831">{{cite journal |vauthors=Hatano T, Yoshino Y, Kawashima Y, Shirai H, Iizuka N, Miyazawa Y, Sakata A, Onishi T |title=Case of gonadoblastoma in a 9-year-old boy without physical abnormalities |journal=Int. J. Urol. |volume=6 |issue=3 |pages=164–6 |date=March 1999 |pmid=10226831 |doi= |url=}}</ref>
* Since this tumor affects [[gonad]]<nowiki/>s, there is no [[genotype]]-[[phenotype]] correlations.
* Since this tumor affects [[gonad]]<nowiki/>s, there is no [[genotype]]-[[phenotype]] correlations.
* The majority of affected individuals appears to be phenotypically female, however, are [[intersex]].
* The majority of affected individuals appears to be phenotypically female, however, are [[intersex]].
*Although very uncommon, there are reports of gonadoblastoma development in phenotypically and genotypically normal men.
* Although very uncommon, there are reports of gonadoblastoma development in phenotypically and genotypically normal men.


==References==
==References==

Revision as of 19:09, 19 February 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

The prevalence of gonadoblastoma depends on the chromosomal content, presence or absence of mosaicism, gonadal histology, and age of the patient and varies between 15,000 to 30,000 per 100,000 individuals worldwide. The incidence of gonadoblastoma varies according to the presence or absence of Y chromosomal content and age of the individual. Patients with Turner syndrome who have Y chromosomal content either completely or partially may develop gonadoblastoma with an incidence as high as 43,000 per 100,000 individuals worldwide. The incidence of gonadoblastoma among phenotypical females with XY gonadal abnormalities have been observed to be 40,000 per 100,000 individuals worldwide. Gonadoblastoma may be found at any age, but commonly presents before the age of 15 years. Since, it usually affects individuals with gonadal dysgenesis, there is no genotype-phenotype correlation. Phenotypically, it tends to affect female individuals to a greater extent.


Epidemiology and Demographics

Incidence

  • The incidence of gonadoblastoma varies according to the presence or absence of Y chromosomal content and age of the individual.[1][2]
  • The incidence of gonadoblastoma among phenotypical females with XY gonadal abnormalities have been observed to be 40,000 per 100,000 individuals worldwide.
  • Patients with Turner syndrome who have Y chromosomal content either completely or partially can develop gonadoblastoma with an incidence as high as 43%.

Prevalence

  • The prevalence of gonadoblastoma depends on the chromosomal content, presence or absence of mosaicism, gonadal histology and age of the patient and varies between 15,000 to 30,000 per 100,000 individuals worldwide.[3]

Age

  • Gonadoblastoma affects individuals of any age but commonly presents before 15 years of age.[4]

Race

  • There is no racial predilection to gonadoblastoma.

Gender

  • It commonly originated from dysgenetic gonad but has been observed in women with normal ovary and karyotypes, although rare.[4][5]
  • Since this tumor affects gonads, there is no genotype-phenotype correlations.
  • The majority of affected individuals appears to be phenotypically female, however, are intersex.
  • Although very uncommon, there are reports of gonadoblastoma development in phenotypically and genotypically normal men.

References

  1. Brant WO, Rajimwale A, Lovell MA, Travers SH, Furness PD, Sorensen M, Oottamasathien S, Koyle MA (May 2006). "Gonadoblastoma and Turner syndrome". J. Urol. 175 (5): 1858–60. doi:10.1016/S0022-5347(05)0032-8. PMID 16600779.
  2. Sperling, M (2014). Pediatric endocrinology. Philadelphia, PA: Elsevier/Saunders. ISBN 978-1-4557-4858-7.
  3. "Yen & Jaffe's Reproductive Endocrinology | ScienceDirect".
  4. 4.0 4.1 Scully RE (1970). "Gonadoblastoma. A review of 74 cases". Cancer. 25 (6): 1340–56. PMID 4193741.
  5. Hatano T, Yoshino Y, Kawashima Y, Shirai H, Iizuka N, Miyazawa Y, Sakata A, Onishi T (March 1999). "Case of gonadoblastoma in a 9-year-old boy without physical abnormalities". Int. J. Urol. 6 (3): 164–6. PMID 10226831.

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