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
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 .
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 testicle , but data is limited
Treatment and prognosis
Although a gonadoblastoma itself is benign, if it is not removed, it tends to develop into a germ cell tumor . They are often discovered coexisting with malignancy. If there is a contralateral undescended testicle, it is often removed, as it is considered likely to also harbor a gonadoblastoma .