Appendiceal Cancer - Early Signs, Risk Factors, Diagnosis, and Treatment Explained

Appendiceal cancer is a rare cancer that starts in the appendix---a small pouch attached to the large intestine. It affects only 1--2 people in a million each year, so most people have never heard of it until diagnosis. Because of its rarity, many people are not aware of it until they or a loved one is diagnosed.

Early detection is important, as appendiceal cancer may not cause symptoms in the beginning and is often found by chance during surgery for appendicitis or other abdominal problems. When detected early, treatment is more effective and survival outcomes are generally better.

What Are the Types of Appendiceal Cancer?

Appendiceal cancer is not just one disease. There are several subtypes, each with unique characteristics. The most common include:

• Carcinoid (Neuroendocrine) Tumors: Slow-growing tumors that develop from hormone-producing cells in the appendix. These are the most common type.
• Mucinous Adenocarcinoma: Produces mucus and can spread within the abdominal cavity, leading to a condition called pseudomyxoma peritonei (PMP).
• Non-Mucinous Adenocarcinoma: Resembles colon cancer and is usually more aggressive.
• Goblet Cell Adenocarcinoma (Adenocarcinoid): A rare tumor with features of both carcinoid and adenocarcinoma, often more aggressive.
• Signet Ring Cell Adenocarcinoma: A very rare, aggressive form that is more difficult to treat.

Understanding the type of appendiceal cancer is crucial, as it guides the treatment plan and helps predict prognosis.

What Are the Causes of Appendiceal Cancer?

The exact cause of appendiceal cancer is still unclear. However, like other cancers, it develops when cells in the appendix undergo changes (mutations) that cause uncontrolled growth. Over time, these abnormal cells may form a tumor and, in some cases, spread to other parts of the body. Doctors do not yet know the exact cause. It may be linked to changes in genes, long-term irritation of the appendix, or lifestyle factors --- but more research is needed.

What Are the Risk Factors of Appendiceal Cancer?

Because appendiceal cancer is rare, clear risk factors are still being studied. However, some possible risk factors include:

• Age: Most cases are diagnosed in people over 50, though it can occur at any age.
• Gender: Some types, like carcinoid tumors, are more common in women.
• Genetics: A family history of gastrointestinal cancers may slightly increase risk.
• Other Medical Conditions: People with conditions like multiple endocrine neoplasia type 1 (MEN1) may have a higher risk.
• Lifestyle Factors: While not proven, factors that increase risk for other digestive system cancers, such as smoking, obesity, and low-fiber diets, may also play a role.

What Are the Symptoms of Appendiceal Cancer?

Early appendiceal cancer often causes no symptoms. When symptoms do appear, they may mimic appendicitis or other abdominal conditions. Signs and symptoms include:

• Pain in the lower right side of the abdomen
• Bloating or swelling
• Changes in bowel habits (constipation or diarrhea)
• Unexplained weight loss
• A feeling of fullness
• Advanced Symptoms:
  • A palpable abdominal mass
  • Ascites (fluid buildup in the abdomen)
  • Widespread abdominal pain
  • Gastrointestinal bleeding (in rare cases)

Because these symptoms are non-specific, appendiceal cancer is often found incidentally during surgery or imaging for another condition.

How Is Appendiceal Cancer Diagnosed?

If a doctor suspects appendiceal cancer, several tests may be done to confirm the diagnosis:

• Imaging Tests: CT scans, MRI, or ultrasound provide detailed pictures of the appendix and surrounding tissues, helping to identify tumors and any signs of cancer spread.
• Blood Tests: Blood tests can measure special proteins called tumor markers (like CEA and CA 19-9). These are not specific for appendix cancer but can help doctors in diagnosis and follow-up.
• Biopsy: A small sample of tissue is removed and examined under a microscope by a pathologist. Performing a biopsy directly on the appendix can be difficult, so biopsies are often taken from other areas, such as the peritoneum (the lining of the abdomen), if the cancer is suspected to have spread.
• Following Surgery: In many cases, diagnosis is made after the appendix is removed for suspected appendicitis.

Staging of Appendiceal Cancer

Once diagnosed, doctors determine the stage and grade of cancer to guide treatment.

Staging describes how far the cancer has spread:

• Stage I: Cancer is confined to the appendix.
• Stage II: Cancer has grown into nearby tissues but not spread further.
• Stage III: Cancer has spread to nearby lymph nodes.
• Stage IV: Cancer has spread to distant organs (metastasis), often within the abdominal cavity.

Grading refers to how abnormal the cancer cells look under a microscope:

• Low-grade: Cells look more like normal tissue; usually grow slowly.
• High-grade: Cells look very abnormal; tend to grow and spread faster.

What Are the Treatment Options for Appendiceal Cancer?

Treatment depends on the type, stage, and grade of the cancer, as well as the patient's overall health.

Surgery

• Appendectomy: Removal of the appendix, often enough for small, localized carcinoid tumors.
• Right Hemicolectomy: Removal of part of the colon and nearby lymph nodes, usually for adenocarcinomas.
• Cytoreductive Surgery with HIPEC (Hyperthermic Intraperitoneal Chemotherapy): In some advanced cases, doctors perform a special operation called cytoreductive surgery followed by HIPEC. In this, after removing visible tumors, heated chemotherapy medicine is placed directly inside the abdomen to kill any remaining cancer cells.

Medical Treatments

• Chemotherapy: Drugs that kill or slow the growth of cancer cells. May be given intravenously or directly into the abdominal cavity (HIPEC).
• Targeted Therapy: Medications designed to attack specific genetic changes in cancer cells.

Radiation Therapy: Rarely used, but may help in specific cases where the cancer has spread or to relieve symptoms.

What Is the Prognosis of Appendiceal Cancer?

The outlook for appendiceal cancer depends on the type, stage, and grade:

• Carcinoid tumors generally have an excellent prognosis, with 5-year survival rates over 90%.
• Adenocarcinomas and signet ring cell cancers are more aggressive, with survival rates ranging from 20--60% depending on stage.
• Pseudomyxoma peritonei can often be managed successfully with surgery and HIPEC, improving survival outcomes.

Factors that influence prognosis include tumor size, spread, patient age, and overall health.

Screening and Prevention of Appendiceal Cancer

There are no official screening guidelines for appendiceal cancer due to its rarity. However, individuals with a family history of gastrointestinal cancers or genetic syndromes may benefit from regular health checks and genetic counselling. Additionally, maintaining a healthy lifestyle, eating a balanced diet, regular exercise, avoiding smoking and alcohol, and managing weight may reduce the risk of gastrointestinal cancers in general.

For International Patients

People from around the world come to Apollo Hospitals for the treatment of appendiceal cancer. Our international patient services team will guide you all the way from seeking the first virtual connect all the way to treatment in India and then returning home post treatment.

Services include:

• Medical opinions and scheduling
  • Pre-arrival medical review of reports and imaging.
• Travel and logistics
  • Assistance with visa invitation letters, airport transfers, and nearby accommodation options.
  • Dedicated international patient coordinators to guide through each step.
• Language and cultural support
  • Interpreter services in multiple languages.
  • Clear, simple explanations at every stage with written care plans.
• Financial coordination
  • Transparent treatment estimates and packages when possible.
  • Support with international payment methods and insurance coordination.
• Continuity of care
  • Shared records, imaging, and treatment summaries for home doctors.
  • Telemedicine follow-ups for convenience after returning home.

Frequently Asked Questions (FAQs)

1. What is the survival rate for appendiceal cancer?

Survival rates vary widely depending on the type and stage. Carcinoid tumors have excellent survival rates (over 90%), while more aggressive forms like signet ring cell cancer have lower survival outcomes.

2. Can appendiceal cancer be cured?

Yes, many cases can be cured, especially if detected early and treated surgically. Advanced cases may require more complex treatment but can still be managed effectively.

3. What are the treatment side effects?

Side effects depend on the treatment. Surgery may cause pain and recovery time. Chemotherapy can cause fatigue, nausea, hair loss, and low immunity. Targeted therapies and immunotherapies may have fewer but specific side effects.

4. How long is recovery after surgery?

Recovery depends on the type of surgery. An appendectomy may require 1--2 weeks, while more extensive surgeries like HIPEC may require several weeks of recovery.

5. Does appendiceal cancer recur?

Yes, recurrence can happen, especially in advanced or aggressive types. Regular follow-ups and imaging are essential after treatment.

6. What is the cost of appendiceal cancer treatment?

Costs vary by country, hospital, and treatment type. In India, surgery and HIPEC are generally more affordable compared to Western countries. Apollo Hospitals provides cost-effective packages for international patients.

7. Is appendiceal cancer the same as colon cancer?

No. While they may share some similarities, appendiceal cancer arises specifically in the appendix and requires different treatment approaches.