Malignant ovarian stromal tumors are rare and represent approximately 1.2% of all primary malignant ovarian tumors. In contrast to epithelial and germ cell tumors, however, sex cord-stromal tumors frequently present with signs of hormonal production, such as hirsutism and virilization, menstrual changes, or early puberty as well as signs and symptoms of a pelvic mass, and are often found in adolescents and young adults, with the exception of adult granulosa cell tumors which typically occur later. Ovarian stromal tumors are often found early, and have a 75% survival rate.
Symptoms may include:
- abnormal uterine bleeding (ovarian stromal tumors sometimes produce estrogen);
- in young girls, can cause menstrual periods and breast development prior to puberty;
- less commonly, these tumors can produce testosterone, which can result in cessation of menstrual periods, as well as facial and body hair growth;
- endometrial hyperplasia;
- abdominal distention/bloating;
- sudden, severe abdominal pain can occur if the tumor starts to bleed, or if ovarian torsion occurs.
Several subtypes are associated with genetic predisposition, including those observed in patients with Peutz-Jegher syndrome. Recent studies have revealed the relationship between Sertoli-Leydig cell tumors and DICER1 mutations. Mutations in DICER1, STK11, and FOXL2 influence the development of some of these neoplasms.
Granulosa cell tumors are more often malignant than thecomas or fibromas, which are most often benign. Granulosa cell, theca cell, and mixed tumors are usually hormonally active, in contrast to fibromas, which do not produce hormones.
- Fibromas (most common granulosa-stromal tumors)
- Adult granulosa cell tumor
- Juvenile granulosa cell tumor
- Sertoli-Leydig cell tumor
- Granulosa and Sertoli-Leydig elements
- Granulosa and Sertoli elements
Currently, individuals with sex cord-stromal tumors receive treatment that is similar to that in individuals with germ cell or epithelial tumors. As translational efforts advance, it is likely that treatment will evolve and that therapies directed at the underlying genetic aberrations may replace more generic treatment. Recurrent disease may be treated with surgery. Radiation therapy may be useful in settings of recurrent disease.