Signet Ring Cell Carcinoma

What is signet ring cell carcinoma?

Signet ring cell carcinoma (SRCC) is a cancer that most commonly starts in your gastrointestinal (GI) tract, especially your stomach. It gets its name from how the cells look underneath a microscope — round but with one side extended, like a signet ring. SRCC is a type of adenocarcinoma, which means the cancer forms from glandular cells. These are normal cells found in the glands that line your organs.

SRCC can be a difficult diagnosis to receive because it’s an aggressive (fast-growing) cancer that requires swift treatment. The challenge is that researchers are still learning which treatments work best. You’ll work closely with your oncologist to find the best care plan for you.

Types of signet ring cell carcinoma

Healthcare providers classify SRCC based on the organ where the cancer starts. Approximately 90% of signet cell ring carcinoma is stomach cancer. Less often, SRCC starts in other parts of your body with glandular cells.

For example, SRCC accounts for 1% or less of the following cancer types:

• Colon cancer.
• Rectal cancer.
• Pancreatic cancer.
• Bladder cancer.
• Gallbladder cancer.
• Esophageal cancer.
• Appendix cancer.
• Prostate cancer.
• Breast cancer.

How common is signet ring cell carcinoma?

Most stomach cancers (up to 95%) are adenocarcinomas. Signet ring cell carcinoma accounts for 35% to 40% of stomach cancers that are adenocarcinomas.

Stomach cancer is the fifth most common cancer in the world, but fewer people are developing it. This is likely because of new treatments that can prevent common stomach cancer risk factors, like Helicobacter pylori (H. pylori) infections. But the number of people diagnosed with stomach cancers that are SRCC is on the rise. Scientists aren’t sure why.

What are the symptoms of signet ring cell carcinoma?

Symptoms of signet ring cell carcinoma usually don’t show up until the cancer has spread. So, most people don’t realize there’s an issue at first. SRCC in your stomach usually spreads within your abdominal cavity (peritoneum), bones and ovaries.

Symptoms of SRCC may include:

• Indigestion.
• Abdominal pain.
• Nausea and vomiting.
• Diarrhea.
• Bloody stools.
• Fatigue.
• Joint pain.
• Pallor (paleness, fast heartbeat and trouble breathing).

Most of these symptoms are also common in gastrointestinal (GI) conditions that are much less serious than cancer. So, it’s important not to assume that cancer is causing the symptoms if you’re experiencing some of them. Still, it’s always a good idea to visit a healthcare provider if you’re experiencing symptoms that don’t improve.

What causes signet ring cell carcinoma?

Signet ring cell carcinoma forms when an abnormal glandular cell makes copies of itself. These abnormal clones can form tumors, spread and destroy healthy tissue. But researchers don’t know what causes the cells associated with SRCC to become abnormal in the first place.

Some studies show that changes to the gene involved in making an important protein in glandular cells (CDH 1) may play a role. But there’s still a lot researchers don’t know yet about how glandular cells become malignant (cancerous) in SRCC.

How is signet ring cell carcinoma diagnosed?

SRCC diagnosis often begins with a healthcare provider reviewing your symptoms and doing a physical exam. This gives them an idea of where the problem may be located within your body. And it helps them decide which tests to do. Tests include:

• Imaging tests: A computed tomography (CT) scan is the most common imaging procedure providers use to diagnose tumors in your GI tract. If your provider finds a suspicious mass that may be a tumor, you may need a PET scan to check for signs that cancer cells have spread.
• Blood tests: Your provider will likely take a blood sample. They may perform several tests, including those that check your general health and those that check for signs of disease, like cancer.
• Biopsy: Your provider will need to remove the entire tumor or a tissue sample and test it to confirm it is SRCC. A pathologist will use a microscope to look for the signet ring cells. They may also perform a test on the biopsy called immunohistochemistry that can show if the cells are cancerous.

How is signet ring cell carcinoma treated?

Your treatment depends on lots of factors that you’ll discuss with your healthcare provider, including its type (where the cancer started) and how advanced it is (its stage). Most people with signet ring cell carcinoma need a combination of treatments, which may include:

• Endoscopic surgery: If the cancer hasn’t spread, surgery may involve a noninvasive procedure like an esophagogastroduodenoscopy. Instead of making cuts to access the tumor, your provider inserts a scope through your mouth that they’ll use to remove the tumor.
• Radical resection: This type of surgery is more common with SRCC, as the cancer has usually spread by the time providers diagnose it. Your provider will remove the tumor and all nearby tissue where the cancer has spread. Providers often remove some lymph nodes, too, as SRCC often spreads there.
• Hyperthermic intraperitoneal chemotherapy (HIPEC): HIPEC uses surgery and chemotherapy (cancer-killing drugs) to treat cancers that have spread within your abdomen. After your provider removes your tumor, they’ll place chemotherapy drugs directly into your abdomen. Then, they’ll heat the drugs. The heat makes chemo especially toxic to any remaining cancer cells.
• Systemic chemotherapy: Your provider may recommend systemic chemotherapy. You’ll take a pill or receive an infusion (IV) of chemo drugs that circulate throughout your body to kill cancer cells. Chemotherapy is a common SRCC treatment, but it’s not effective long term.
• Radiation therapy: Some providers recommend that people get radiation therapy and chemotherapy after surgery to kill any remaining cancer cells. The type of radiation treatment for SRCC uses a machine outside of your body to direct energy toward cancer cells.
• Clinical trials: Clinical trials test the safety and effectiveness of new cancer treatments. Scientists continue to study the cell changes that lead to SRCC, with the goal of developing treatments that can stop these changes. Taking part in a clinical trial may provide access to these new treatments.

What can I expect if I have SRCC?

There isn’t any way to predict what anyone’s unique experience with signet ring cell cancer will be like. But we do know that SRCC is an especially aggressive cancer when compared to other carcinomas. It often returns (recurs) after surgery, and it’s difficult to manage long term with standard cancer treatments like chemotherapy.

In many ways, your experience will depend on your goals. If your cancer is in the early stages, sending and keeping the cancer in remission (no signs or symptoms) may be a goal. With cancer that’s spread, including Stage 4 signet ring cell cancer, treatments may help you live more comfortably.

Ask your healthcare provider about what to expect based on your diagnosis.

How do I take care of myself?

It’s important to learn as much as you can about your SRCC diagnosis. It’s better to get answers (even hard ones) than experience anxiety over what’s happening in your body. Ask your healthcare provider what your diagnosis means for your overall health and daily routine.

Also, ask your healthcare provider if you could benefit from palliative care resources. The goal of palliative care is to provide symptom relief. Regardless of your cancer stage, these resources may provide relief from cancer symptoms and treatment side effects.

When should I see my healthcare provider?

See a healthcare provider if you’re experiencing ongoing, unexplained GI symptoms, like belly pain or diarrhea. Usually, these symptoms are a sign of less serious conditions. In extremely rare cases, they may be signs of a tumor in your abdominal cavity.

When should I go to the ER?

Go to the ER if you’re experiencing symptoms like dizziness, fever or shortness of breath (dyspnea). These symptoms are common with a lot of conditions that require emergency care, including advanced signet ring cell carcinoma.

What questions should I ask my healthcare provider?

It’s important to understand all that your diagnosis involves, including how it may affect your everyday life. Questions may include:

• What type of cancer do I have?
• What stage is my cancer?
• What treatments would you recommend?
• What treatment side effects can I expect?
• What is my prognosis (likely outcomes with treatment)?
• What steps can I take to make living with SRCC easier?