Germ Cell and Sex Cord Stromal Ovarian Tumors

Germ cell and sex cord stromal ovarian tumors are unusual forms of ovarian ancer. They are quite different from epithelial cell ovarian cancer, the type of cancer most people are referring to when they talk about ovarian cancer.

The ovary is comprised of different types of cells. Germ cells are the reproductive cells, the egg in a female, sperm in a male. The stroma is the tissue that provides structural support and produces various hormones that are necessary to support the reproductive process. Epithelial cells are the covering of the ovary. Any of these cells can become cancerous and producing distinct symptoms and also having different prognoses.

Germ cell tumors can be either benign (non-cancerous) or malignant (cancerous). The most common benign germ cell tumor is a dermoid cyst more accurately called a mature teratoma. These tumors are usually found during the reproductive years but can be found in children or postmenopausal women. They may be detected during a pelvic exam or an incidental finding on a radiological study, occasionally they may cause pain due to their size or if they twist on themselves, a process called ovarian torsion. As they are derived from cells that have the ability to differentiate into every type of cell, the dermoid cysts contain substances such as hair, cartilage and even teeth. A rare type of teratoma called struma ovarii contains thyroid tissue and may present with symptoms of an overactive thyroid.

There are seven distinct types of malignant ovarian germ cell tumors. Each are composed of different types of cells and secrete different substances called tumor markers. In contrast to ovarian epithelial cell cancers germ cell tumors are usually found at an early stage. Because of this and their sensitivity to chemotherapy and radiation the survival rate is much higher. The peak incidence of germ cell tumors is in women in their early twenties. Thus conserving reproductive function is critical when treating of these tumors.

Women with a germ cell tumor may present with abdominal swelling, pain or abnormal bleeding. A mass may be detected during a pelvic exam or imaging studies such as a CAT scan or ultrasound. Specific tumor markers may also be elevated, raising suspicion of a particular type of germ cell tumor. Treatment (and diagnosis) is initially surgical with removal of the affected ovary. The entire abdomen is inspected and samples taken to determine if the tumor has spread anywhere. Removal of the other ovary and uterus is not necessary if the tumor has not spread and the affected woman wishes to preserve her fertility. Whether chemotherapy or radiation is needed depends on the type of tumor and if it has spread.

Ovarian stromal tumors are fairly uncommon tumors arising from the ovarian stromal tissue. These are classified into granulosa or Sertoli-Leydig cell tumors depending on which cells they are derived from. Granulosa cell tumors produce estrogen thus can present with symptoms of estrogen excess such as abnormal bleeding, breast tenderness or even uterine cancer. They have a tendency to rupture causing sudden onset of severe abdominal pain. Treatment is initially surgical with preservation of the other ovary if possible. Overall these tumors have a 75-90% survival rate. Fibromas are another type of stromal cell tumor. Although benign they can be associated with an unusual syndrome that can mimic an advanced cancer. Meigs’ syndrome occurs in the presence of an ovarian fibroma along with fluid in the abdomen and the lungs. Sertoli-Leydig cell tumors may produce large amounts of masculinizing hormones. Women and girls with these tumors may present with rapid onset of defeminization starting with the loss of menstrual periods and progressing to loss of feminine body contour, acne, deepening of the voice, abnormal hair growth or even male pattern balding.

Although uncommon germ cell tumors are always in the differential diagnosis of many reproductive problems. It is always important to seek medical care if abnormal symptoms persist.