OHSU Knight Cancer Institute

Rare Cancers

Dr. Craig Okada walking in a hallway with a patient.

Discovering you have cancer is an emotional experience, especially if your cancer is rare. You may have trouble finding doctors who are familiar with your condition. You may have trouble finding other people who have gone through the same thing. You may even have trouble finding solid information.

You are not alone. The OHSU Knight Cancer Institute has a wealth of experience in caring for people with rare cancers. Here’s what we have to offer:

  • Expert doctors who are leaders in their fields.
  • A team approach, with doctors from different specialties looking at your case together.
  • Clinical trials and promising new treatments.
  • A deep commitment to research, including a center devoted to early detection.
  • A U.S. News & World Report ranking among the best cancer centers in the nation.
  • A full menu of support services for you and your family.

Understanding rare cancers

There are thousands of types of cancer. Some types, like skin cancer, affect millions of Americans every year. Other types are so unusual that fewer than 100 cases have ever been reported.

The National Cancer Institute defines a rare cancer as any type found in fewer than 15 out of 100,000 Americans a year.

Just because a cancer is rare doesn’t necessarily make it deadly. Some rare cancers cause little trouble. Others are aggressive and need prompt attention. Having a rare cancer can pose special problems, however. When you have a rare cancer, it can be harder to find:

  • Doctors who are familiar with your condition.
  • Solid statistics on outcomes.
  • Treatment designed for your condition.
  • Other people going through the same thing.

What makes us different?

Doctors at the Knight have extensive experience treating cancer, especially rare and complex conditions. Our care is based on the latest research, some of it by our own scientists. That means we offer access to new therapies as soon as they become available. Our discoveries and innovations have helped transform the way cancer is treated. Read more about how we're helping the patients of today and tomorrow.

Some of the rare cancers we treat

“We are at the forefront.” Dr. Liana Tsikitis of the Knight Cancer Institute talks about the team that does surgery for appendix cancer and other GI cancers. She is a leading authority on appendix cancer.

Also known as: Appendiceal cancer, appendiceal tumor.

What is it? Appendix cancer is a gastrointestinal (GI) cancer. It grows inside your appendix, a small pouch shaped like a sausage that connects to your colon.

Appendix cancer affects 1 to 2 people per million. Because it is so rare, statistics on survival rates aren’t always solid. In general, the outcome depends on how early it is caught and how far it has spread.

Symptoms:

  • Appendicitis, a painful swelling in the abdomen; most cases are not caused by cancer
  • Bloating
  • Jelly belly, a buildup of fluid in the abdomen

Treatments:

  • Surgery: This is the mainstay for treating appendix cancer. Surgeons may perform an appendectomy, removing your appendix. They may also perform a hemicolectomy, removing a section of colon.
  • Chemotherapy: This is medication that kills cancer cells. The basic idea is simple: The medication attacks cells that divide rapidly, like cancer cells.
  • Hyperthermic intraperitoneal chemotherapy (HIPEC): Surgeons remove your appendix and any tumors that have spread from it. Then they flood your abdominal cavity with chemotherapy medication.

Meet our team of experts who specialize in GI cancer.

Learn more:

What is it? Pseudomyxoma peritonei occurs in the peritoneum, a large cavity in your abdomen containing your stomach, liver and intestines.

This cancer begins with a small tumor in your appendix, a small pouch shaped like a sausage. The tumor grows through the wall of the appendix and erupts into the peritoneum. From there it spreads and creates more tumors. Pseudomyxoma peritonei affects 1 to 2 people per million.

Symptoms:

  • Appendicitis, a painful swelling in the abdomen; most cases are not caused by cancer
  • Bloating
  • Jelly belly, a buildup of fluid in the abdomen

Treatments:

  • Surgery: This is the mainstay for treating appendix cancer. Surgeons may perform an appendectomy, removing your appendix. They may also perform a hemicolectomy, removing a section of colon.
  • Chemotherapy: This is medication that kills cancer cells. The basic idea is simple: the medication attacks cells that divide rapidly, like cancer cells.
  • Hyperthermic intraperitoneal chemotherapy (HIPEC): Surgeons remove your appendix and any tumors that have spread from it. Then they flood your abdominal cavity with chemotherapy medication.

Meet our team of experts who specialize in GI cancer.

Learn more:

What is it? Penile cancer most commonly occurs on the head or foreskin of the penis, but it can occur on other parts, as well.

About 2,000 cases are reported each year in the United States. It is more common in men who have:

  • Been exposed to the human papilloma virus (HPV)
  • Phimosis (difficulty pulling back the foreskin)

Symptoms:

  • A growing sore or lump on the penis, especially on the head or foreskin.
  • Thickening and reddening of the skin on the penis.
  • Enlarged groin lymph nodes.

Treatments:

  • Topical therapy: When caught early, penile cancer can often be treated with skin creams which kill the cancer cells without the need for surgery. The creams may contain chemotherapy medication or immunotherapy medication.
  • Radiation therapy: Radiation therapy uses beams of high-energy particles to destroy cancer cells. It is sometimes combined with surgery or chemotherapy.
  • Surgery: Our surgeons are experts in a variety of techniques to remove penile cancer while preserving as much appearance and function as possible. These include laser surgery, cryosurgery, and Mohs surgery.
  • When removal of lymph nodes is necessary, we use minimally invasive techniques like laparoscopy or robotic surgery to minimize side effects and hasten recovery.
  •  Our surgeons are also experts in reconstructive surgery when this is necessary.

Survivorship:

  • Our goal is not just to eliminate cancer but to restore health and well-being. Our doctors are experts in sexual medicine and work with physical therapists, occupational therapists, sex therapists, and psychologists to help you live well after treatment.

Meet our team of experts who specialize in urologic cancer.

Learn more:

A doctor smiles in warmth and reassurance to a patient in a clinic.
Dr. Craig Okada is an expert on cutaneous lymphoma. He is working on new ways to treat this rare condition.

Also known as: Lymphoma of the skin.

What is it? Lymphoma is a disease of the lymphocytes, a type of white blood cell. Lymphocytes are produced by your bone marrow and travel through your blood, lymph system and other tissues.

In most cases, the cancer cells circulate in the blood or the lymph system. But in some cases, they clump together to form tumors in your skin.

Cutaneous lymphoma has several types. Some grow fast, some slow. Some are highly treatable. Some are aggressive.

Symptoms: Cutaneous lymphoma can be hard to tell apart from other skin inflammations. Signs include:

  • Inflamed, itchy welts or patches
  • Raised bumps or patches
  • Discolored lesions
  • Tumor or mass in or under the skin

Treatments:

  • Skin-directed treatment: When the lymphoma involves smaller areas of the body, doctors often treat it with topical drugs such as steroids, ultraviolet light, surgery, chemotherapy or radiation therapy to destroy tumors.
  • Systemic treatment: When the lymphoma is more widespread, doctors use chemotherapy, immunotherapy and targeted therapy to attack cancer cells throughout the body.
  • Chemotherapy: This is medication that kills cancer cells. Typically the medication damages fast-growing cells, like cancer cells, so that they die.
  • Immunotherapy: Immunotherapy uses your own immune system to destroy cancer cells. Doctors may use medications like rituximab, which locks onto a protein on a cancer cell’s surface, then signals your immune system to destroy the cell.
  • Targeted therapy: Some cancer cells divide uncontrollably because they have a molecular switch that is stuck in the “on” position. Targeted therapy medications take advantage of this by locking onto the switch and turning it off.
  • Radiation therapy: Radiation therapy uses beams of high-energy particles to damage cancer cells so that they die. Radiation is often used when cutaneous lymphoma involves a smaller area of the body.

Meet our team of experts who specialize in lymphoma and blood cancers.

Learn more:

For patients

Call 503-494-7999 to:

  • Request an appointment
  • Seek a second opinion
  • Ask questions

Refer a patient

Targeted therapy stops a rare, joint-destroying tumor

cancer survival story Brian Matekovich

Brian Matakovich was successfully treated with a new drug for a rare type of bone cancer. His doctor at the Knight treated him with pexidartinib, a targeted therapy.

Read more

Learn more about OHSU Knight Cancer Institute treatments and research:

Stay informed

Blog: Learn about the latest findings on our Cancer Translated blog.

News: Read about research breakthroughs, patient care and many other topics on the OHSU News site.