What Is Ovarian Cancer?

The ovaries are mainly made up of 3 kinds of cells. Each type of cell can develop into a different type of tumor:

• Epithelial tumors start from the cells that cover the outer surface of the ovary. Most ovarian tumors are epithelial cell tumors.
• Germ cell tumors start from the cells that produce the eggs (ova).
• Stromal tumors start from structural tissue cells that hold the ovary together and produce the female hormones estrogen and progesterone.

Some of these tumors are benign (non-cancerous) and never spread beyond the ovary. Malignant (cancerous) or borderline (low malignant potential) ovarian tumors can spread (metastasize) to other parts of the body and can be fatal.

Epithelial ovarian tumors

Epithelial ovarian tumors start in the outer surface of the ovaries. These tumors can be benign (not cancer), borderline (low malignant potential), or malignant (cancer).

Benign epithelial ovarian tumors

Epithelial ovarian tumors that are benign don’t spread and usually don’t lead to serious illness. There are several types of benign epithelial tumors including serous cystadenomas, mucinous cystadenomas, and Brenner tumors.

Borderline Epithelial Tumors

When looked at in the lab, some ovarian epithelial tumors don’t clearly appear to be cancerous and are known as borderline epithelial ovarian cancer. The two most common types are atypical proliferative serous carcinoma and atypical proliferative mucinous carcinoma. These tumors were previously called tumors of low malignant potential (LMP tumors). These are different from typical ovarian cancers because they don’t grow into the supporting tissue of the ovary (called the ovarian stroma). If they do spread outside the ovary, for example, into the abdominal cavity (belly), they might grow on the lining of the abdomen but not into it.

Borderline tumors tend to affect younger women than the typical ovarian cancers. These tumors grow slowly and are less life-threatening than most ovarian cancers.

Malignant epithelial ovarian tumors

Cancerous epithelial tumors are called carcinomas. About 85% to 90% of malignant ovarian cancers are epithelial ovarian carcinomas. These tumor cells have several features (when looked at in the lab) that can be used to classify epithelial ovarian carcinomas into different types. The serous type is by far the most common, and can include high grade and low grade tumors. The other main types include mucinous, endometrioid, and clear cell.

• Serous carcinomas (52%)
• Clear cell carcinoma (6%)
• Mucinous carcinoma (6%)
• Endometrioid carcinoma (10%)

Each ovarian cancer is given a grade, based on how much the tumor cells look like normal tissue:

• Grade 1 epithelial ovarian carcinomas look more like normal tissue and tend to have a better prognosis (outlook).
• Grade 3 epithelial ovarian carcinomas look less like normal tissue and usually have a worse outlook.

Other traits are also taken into account, such as how fast the cancer cells grow and how well they respond to chemotherapy, to come up with the tumor's type:

• Type I tumors tend to grow slowly and cause fewer symptoms. These tumors also seem not to respond well to chemotherapy. Low grade (grade 1) serous carcinoma, clear cell carcinoma, mucinous carcinoma and endometrioid carcinoma are examples of type I tumors.
• Type II tumors grow fast and tend to spread sooner. These tumors tend to respond better to chemotherapy. High grade (grade 3) serous carcinoma is an example of a type II tumor.

Other cancers that are similar to epithelial ovarian cancer

Primary peritoneal carcinoma

Primary peritoneal carcinoma (PPC) is a rare cancer closely related to epithelial ovarian cancer. At surgery, it looks the same as an epithelial ovarian cancer that has spread through the abdomen. In the lab, PPC also looks just like epithelial ovarian cancer. Other names for this cancer include extra-ovarian (meaning outside the ovary) primary peritoneal carcinoma (EOPPC) and serous surface papillary carcinoma.

PPC appears to start in the cells lining the inside of the fallopian tubes.

Like ovarian cancer, PPC tends to spread along the surfaces of the pelvis and abdomen, so it is often difficult to tell exactly where the cancer first started. This type of cancer can occur in women who still have their ovaries, but it is of more concern for women who have had their ovaries removed to prevent ovarian cancer. This cancer does rarely occur in men.

Symptoms of PPC are similar to those of ovarian cancer, including abdominal pain or bloating, nausea, vomiting, indigestion, and a change in bowel habits. Also, like ovarian cancer, PPC may elevate the blood level of a tumor marker called CA-125.

Women with PPC usually get the same treatment as those with widespread ovarian cancer. This could include surgery to remove as much of the cancer as possible (a process called debulking that is discussed in the section about surgery), followed by chemotherapy like that given for ovarian cancer. Its outlook is likely to be similar to widespread ovarian cancer.

Fallopian tube cancer

This is another rare cancer that is similar to epithelial ovarian cancer. It begins in the tube that carries an egg from the ovary to the uterus (the fallopian tube). Like PPC, fallopian tube cancer and ovarian cancer have similar symptoms. The treatment for fallopian tube cancer is much like that for ovarian cancer, but the outlook (prognosis) is slightly better.

Ovarian germ cell tumors

Germ cells usually form the ova or eggs in females and the sperm in males. Most ovarian germ cell tumors are benign, but some are cancerous and may be life threatening. Less than 2% of ovarian cancers are germ cell tumors. Overall, they have a good outlook, with more than 9 out of 10 patients surviving at least 5 years after diagnosis. There are several subtypes of germ cell tumors. The most common germ cell tumors are teratomas, dysgerminomas, endodermal sinus tumors, and choriocarcinomas. Germ cell tumors can also be a mix of more than a single subtype.

Teratoma

Teratomas are germ cell tumors with areas that, when seen under the microscope, look like each of the 3 layers of a developing embryo: the endoderm (innermost layer), mesoderm (middle layer), and ectoderm (outer layer). This germ cell tumor has a benign form called mature teratoma and a cancerous form called immature teratoma.

The mature teratoma is by far the most common ovarian germ cell tumor. It is a benign tumor that usually affects women of reproductive age (teens through forties). It is often called a dermoid cyst because its lining is made up of tissue similar to skin (dermis). These tumors or cysts can contain different kinds of benign tissues including, bone, hair, and teeth. The patient is cured by surgical removal of the cyst, but sometimes a new cyst develops later in the other ovary.

Immature teratomas are a type of cancer. They occur in girls and young women, usually younger than 18. These are rare cancers that contain cells that look like those from embryonic or fetal tissues such as connective tissue, respiratory passages, and brain. Tumors that are relatively more mature (called grade 1 immature teratoma) and haven’t spread beyond the ovary are treated by surgical removal of the ovary. When they have spread beyond the ovary and/or much of the tumor has a very immature appearance (grade 2 or 3 immature teratomas), chemotherapy is recommended in addition to surgery.

Dysgerminoma

This type of cancer is rare, but it is the most common ovarian germ cell cancer. It usually affects women in their teens and twenties. Dysgerminomas are considered malignant (cancerous), but most don’t grow or spread very rapidly. When they are limited to the ovary, more than 75% of patients are cured by surgically removing the ovary, without any further treatment. Even when the tumor has spread further (or if it comes back later), surgery, radiation therapy, and/or chemotherapy are effective in controlling or curing the disease in about 90% of patients.

Endodermal sinus tumor (yolk sac tumor) and choriocarcinoma

These very rare tumors typically affect girls and young women. They tend to grow and spread rapidly but are usually very sensitive to chemotherapy. Choriocarcinoma that starts in the placenta (during pregnancy) is more common than the kind that starts in the ovary. Placental choriocarcinomas usually respond better to chemotherapy than ovarian choriocarcinomas do.

Ovarian stromal tumors

About 1% of ovarian cancers are ovarian stromal cell tumors. More than half of stromal tumors are found in women older than 50, but about 5% of stromal tumors occur in young girls.

The most common symptom of these tumors is abnormal vaginal bleeding. This happens because many of these tumors produce female hormones (estrogen). These hormones can cause vaginal bleeding (like a period) to start again after menopause. In young girls, these tumors can also cause menstrual periods and breast development to occur before puberty.

Less often, stromal tumors make male hormones (like testosterone). If male hormones are produced, the tumors can cause normal menstrual periods to stop. They can also make facial and body hair grow. If the stromal tumor starts to bleed, it can cause sudden, severe abdominal pain.

Types of malignant (cancerous) stromal tumors include granulosa cell tumors (the most common type), granulosa-theca tumors, and Sertoli-Leydig cell tumors, which are usually considered low-grade cancers. Thecomas and fibromas are benign stromal tumors. Cancerous stromal tumors are often found at an early stage and have a good outlook, with more than 75% of patients surviving long-term.

Ovarian cysts

An ovarian cyst is a collection of fluid inside an ovary. Most ovarian cysts occur as a normal part of the process of ovulation (egg release) -- these are called functional cysts. These cysts usually go away within a few months without any treatment. If you develop a cyst, your doctor may want to check it again after your next menstrual cycle (period) to see if it has gotten smaller.

An ovarian cyst can be more concerning in a female who isn't ovulating (like a woman after menopause or a girl who hasn't started her periods), and the doctor may want to do more tests. The doctor may also order other tests if the cyst is large or if it does not go away in a few months. Even though most of these cysts are benign (not cancer), a small number of them could be cancer. Sometimes the only way to know for sure if the cyst is cancer is to take it out with surgery. Cysts that appear to be benign (based on how they look on imaging tests) can be observed (with repeated physical exams and imaging tests), or removed with surgery.