About malignant appendical tumor

What is malignant appendical tumor?

Pseudomyxoma peritonei is a rare malignant growth characterized by the progressive accumulation of mucus-secreting (mucinous) tumor cells within the abdomen and pelvis. The disorder develops after a small growth (polyp) located within the appendix bursts through the wall of the appendix, and spreads mucus-producing tumor cells throughout the surrounding surfaces (e.g., the membrane that lines the abdominal cavity [peritoneum]). As mucinous tumor cells accumulate, the abdominal area becomes swollen and digestive (gastrointestinal) function becomes impaired. Pseudomyxoma peritonei develops at a variable rate, but may grow at a slower rate (indolent) than other malignancies within the abdomen.

What are the symptoms for malignant appendical tumor?

More than half of people with appendix cancer don’t have symptoms. It’s usually discovered during surgery or an imaging test for another condition like appendicitis.

Your doctor may also discover it during a routine colonoscopy. However, if there are symptoms, they may include:

Many of these symptoms may not occur until the cancer is more advanced. In about a third of cases, the cancer has already spread to distant parts of your body by the time of diagnosis.

What are the causes for malignant appendical tumor?

The exact cause of appendiceal cancer is unknown. There are no genetic, familial or environmental factors known to cause this disorder. It does not run in families. One study has shown a correlation with the presence of the intestinal bacterium Helicobacter pylori and pseudomyoma peritonei from appendiceal neoplasms. This has prompted a clinical trial using antibiotic therapy to eradicate the bacteria. This study is still on-going at the time of this report. Recent studies have shown that appendiceal cancer do have a unique genomic profile distinct from adenocarcinomas of the colon which may offer future, appendix-specific and targetable pathways for treatment.

What are the treatments for malignant appendical tumor?

The treatment for appendix cancer depends on the:

  • type of tumor
  • stage of the cancer
  • person’s overall health

A multidisciplinary team of medical professionals will help you through your treatment. Your team will include a variety of professionals that may include doctors, nurse practitioners, dietitians, counselors, and more. A type of doctor called a surgical oncologist will operate on your cancer while a medical oncologist will develop your chemotherapy plan.


Surgery is the most common treatment for localized appendix cancer. If the cancer is localized to the appendix only, the treatment is usually to remove the appendix. This is also called an appendectomy.

For some types of appendix cancer, or if the tumor is larger, your doctor may recommend removing one-half of your colon and also some lymph nodes. Surgery to remove half of your colon is called a hemicolectomy.

If the cancer has spread, your doctor may recommend cytoreductive surgery, also called debulking. In this type of surgery, the surgeon will remove the tumor, surrounding fluid, and possibly any nearby organs that are attached to the tumor.


Treatment may include chemotherapy before or after surgery if:

  • the tumor is larger than 2 centimeters
  • the cancer has spread, especially to the lymph nodes
  • the cancer is more aggressive

Types of chemotherapy include:

  • systemic chemotherapy given intravenously or by mouth
  • regional chemotherapy given directly into the abdomen, such as intraperitoneal chemotherapy (EPIC) or hyperthermic intraperitoneal chemotherapy (HIPEC)
  • a combination of systemic and regional chemotherapies
Radiation therapy

Radiation therapy is rarely used to treat appendix cancer. However, it may be recommended if your cancer spreads to other body parts.

Imaging tests

After surgery, your doctor will follow up with imaging tests, such as a CT scan or MRI, to ensure the tumor is gone.

What’s the recurrence and survival rate for appendix cancer?

Since appendix cancer is such a rare condition, there’s little information available about its recurrence or survival rates.

According to the American Society of Clinical Oncology, , the 5-year survival rate for Grade 1 and Grade 2 neuroendocrine tumors is between 67 to 97 percent. The survival rate for advanced appendix cancer that has spread to other body parts is lower.

Survival rates also vary between cancer types. Neuroendocrine tumors have the highest chance of survival while signet-ring cell tumors have the lowest five-year survival rate at 27 percentTrusted Source.

The 5-year survival rate increases for some cases of appendix cancer when part of the colon is also removed and chemotherapy is used. However, not all cases of appendix cancer require these additional treatments.


The survival rate and outlook are generally good for most people with early-stage appendix cancer.

In most cases, appendix cancer goes undetected until an appendectomy is already being performed for other reasons.

After any cancer diagnosis, it’s important to follow up regularly with your doctor to be sure there’s no recurrence of cancer.

What are the risk factors for malignant appendical tumor?

The cause of appendix cancer is largely unknown and no avoidable risk factors have been identified. However, appendix cancer becomes more common with age and is rare in children.

Neuroendocrine tumors are more common in women than men.

Although more research is needed, a few potential risk factors have been suggested by experts, including:

  • pernicious anemia, a deficiency of vitamin B12
  • atrophic gastritis, or long-term inflammation of the stomach lining
  • Zollinger-Ellison syndrome, a condition of the digestive tract
  • a family history of multiple endocrine neoplasia type 1 (MEN1), a disorder that leads to tumors in the glands that produce hormones
  • smoking

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