Rare Ovarian Cancer Diagnosis: What Does It Mean?

“You’ve got cancer,” are among the words no one wants to hear - ever. Perhaps even more hard to hear can be, “you’ve got a rare form of cancer,” or “we think you have cancer, but we’re not sure what type.” A diagnosis of a rare ovarian cancer can feel shocking and scary.

What are rare cancers?

There is no single definition of rare cancers. Overall, they account for 20 percent of all cancers, but impact a relatively small number of people compared to the more common cancers. Diseases are classified as rare as in the United States when they affect less than 1 in 200,000 people at any one time.

Most rare diseases have genetic origins. About 20 percent are the result of infections, allergies, or environmental causes. Rare cancers may be degenerative (the body increasingly deteriorates over time) or proliferative (they multiply or grow quickly).

Rare cancers at any age are often misdiagnosed and poorly treated. This happens when test results are misinterpreted and treatment set based on a poor understanding and limited medical expertise. The reality is there are often no effective cures for rare cancers, even when they are correctly diagnosed.

Common types of ovarian cancer

The ovaries are made of 3 main types of cells. Each cell type can develop into a different kind of tumor including epithelial tumors, germ cell tumors, and stromal tumors.¹ Epithelial ovarian cancer, which develops on the surface of the ovary (known as the epithelium), is the most common.³

Epithelial ovarian tumors

The majority of ovarian cancers are epithelial tumors and make up 85 to 90 percent of ovarian cancers.^1,2 There are different types of cancerous epithelial ovarian tumors and serous carcinomas are the most common subtype.^1,2

Fallopian tube cancer (FTC) and primary peritoneal carcinoma (PPC) are other types of cancer that are often grouped together with epithelial ovarian cancer. FTC and PPC are rare cancers, but their symptoms, disease spread, and treatment are similar to ovarian cancer.²

Rare types of ovarian cancer

Germ cell ovarian tumors

Ovarian germ cell tumors grow from the reproductive cells of the ovaries (known as eggs). Most ovarian germ cell tumors are not cancerous. Between 2 and 5 percent of ovarian cancers are germ cells tumors.^1,3 Cancerous germ cell tumors generally occur in teenage girls and women in their 20s.²

Stromal ovarian tumors

Ovarian stromal tumors develop from the connective tissue cells that hold the ovary together. These tumors are very rare and account for only 1 percent of ovarian cancers.^1,2

Small cell carcinoma of the ovary (SCCO)

Small cell carcinoma of the ovary (SCCO) is an extremely rare form of ovarian cancer that accounts for less than 1 percent of ovarian cancers.³ SCCO is mainly diagnosed in younger women. It is currently unknown whether SCCO develops from epithelial cells, stromal cells, or germ cells.

Research for rare cancers

There is less research conducted on rare cancers and there is less financial support for drug research. The Orphan Drug Act of 1983 encourages research for the improvement of treatments for rare cancers.⁴ Low prevalence and difficulty in diagnosis or misdiagnosis by doctors can pose challenges to supporting the development of new drugs for ovarian cancers.

Challenges finding information

There are resources available for people diagnosed with all kinds of diseases. However, people with rare conditions often find it harder to identify reliable sources for information. You may find that doctors, nurses, and even other cancer patients are unfamiliar with your type of cancer.

Consider getting a referral to a hospital cancer center where there are specialists with experience treating the type cancer type you have. The National Cancer Institute (NCI) lists designated cancer centers on the NCI website or you can call 1-800-4-CANCER. You might want to get a second opinion on the diagnosis you received and any recommended treatment plans.⁵

Support resources for rare cancers

Everyone reacts differently to finding out they have cancer. Learning you are diagnosed with a rare type of ovarian cancer can be overwhelming and scary. Family and friends can play a valuable role by providing physical, practical, and emotional support.

There are networks and resources devoted to research and support, specifically for people with rare cancers. AdvancedOvarianCancer.net is a community where you can find educational information, share experiences or learn about the personal journeys of others with all types of ovarian cancer.

Other resources where you can seek information on rare ovarian cancers include:

• American Cancer Society
• International Rare Cancer Initiative
• National Cancer Institute
• Ovarian Cancer Research Alliance

Remember, you are never alone

Research is ongoing to find better ways to prevent, screen for, diagnose, or treat rare diseases. Clinical trials are an essential part of cancer research. These may discover treatment alternatives for those for whom standard and approved treatments do not work. Ask questions of your medical team, discuss treatment options, and explore clinical trials. A rare ovarian cancer diagnosis poses unique challenges, but remember that you are not alone in your cancer journey.