Gonadoblastoma epidemiology and demographics

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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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