Sunday, January 5, 2014

Gonadoblastoma

A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements,  such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells.


High magnification micrograph of a gonadoblastoma, a rare tumour of the gonads that is composed of a sex cord-stromal element (Sertoli cells or granulosa cells) and primitive germ cells. H&E stain.

Gonadoblastoma - very low mag.jpg

Associations

Gonadoblastoma is most often associated with abnormal chromosomal karyotype, gonadal dysgenesis, or the presence of a Y chromosome in over 90% of cases. It has been found in association with Turner's syndrome.

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-oopherectomy (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.

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