Gonadoblastoma

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Gonadoblastoma
Gonadoblastoma - b - high mag.jpg
Micrograph of a gonadoblastoma. H&E stain.
Classification and external resources
Specialty Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value).
ICD-O M9073/1
DiseasesDB 34299
Patient UK Gonadoblastoma
MeSH D018238
[[[d:Lua error in Module:Wikidata at line 863: attempt to index field 'wikibase' (a nil value).|edit on Wikidata]]]

A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements,[1] such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells. Most gonadoblastomas are benign.[2]

Associations

Gonadoblastoma is most often associated with an abnormal chromosomal karyotype, gonadal dysgenesis, or the presence of a Y chromosome in over 90% of cases. Gonadoblastoma has been found in association with androgen insensitivity syndrome, mixed gonadal dysgenesis and Turner syndrome, especially in the presence of Y chromosome-bearing cells.[3][4]

Classification

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Gonadoblastomas can contain elements of both germ cells and gonadal stroma.[5]

Formerly, gonadoblastoma was sometimes regarded as a subset of dysgerminoma. In modern literature, it is sometimes considered to progress to dysgerminoma.[6]

Treatment

Standard treatment would include surgical exploration via laparotomy. Laparoscopy may be an option if the surgeon is particularly skilled in removing ovarian neoplasms via laparoscopy intact. If the diagnosis of gonadoblastoma is certain, a bilateral salpingo-oophorectomy (BSO) should be performed to remove both the primary tumor and the dysgenic contralateral ovary. If uninvolved, the uterus should be left intact. Modern reproductive endocrinology technology allows patients post BSO to achieve pregnancy via in-vitro fertilization (IVF) with a donor egg.

References

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  2. Shahrzad Ehdaivand, Nalini Gupta "Gonadoblastoma", PathologyOutlines.com, 2 July 2014
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  5. Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2009) Robbins & Cotran Pathologic Basis of Disease (8th ed.). Saunders. Chapter 21. ISBN 1-4160-3121-9.
  6. Lua error in package.lua at line 80: module 'strict' not found.

External links