Last revised by Jeremy Jones on 20 Sep 2021

Gonadoblastomas are uncommon sex cord / stromal tumors. They are associated with disorders of sexual development (previously known as "intersex disorders").

The vast majority are found <30 years of age. Most are discovered in the perinatal period. May occur in phenotypic males or females.

  • associated with disorders of sexual development (previously known as "intersex disorders")
  • often found during a workup for a newborn or infant with ambiguous genitalia
  • may be hormonally active and present with precocious puberty or virilization 5

They are associated with chromosomal abnormalities and gonadal dysgenesis.

Gonadoblastomas are considered by some to be a subtype of undifferentiated (or "mixed" sex cord / stromal tumors, although others consider them distinct entities 2.

Other imaging findings of a developmental sex disorder should be present. They are bilateral in 30-50% of cases.

  • imaging findings are not well defined
  • reported as a hyperechoic lesion in the testis 4, but data is limited

Although a gonadoblastoma itself is benign, if it is not removed, it tends to develop into a germ cell tumor 2.  They are often discovered coexisting with malignancy. If there is a contralateral undescended testis, it is often removed, as it is considered likely to also harbor a gonadoblastoma 3.

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