A Silent Threat: Understanding Appendix Cancer and Its Stages

A Silent Threat: Understanding Appendix Cancer and Its Stages

Dr. Devendra Parikh Consultant- GI Surgical Oncology, HCG Aastha Cancer Centre – Ahmedabad

Cancers that arise in a small organ which often remain silent for a long time, making early detection a challenge. Under certain circumstances, even these unnoticed organs can become a source of serious medical conditions.

Among such rare conditions is appendix cancer, which, despite its subtle nature, can have significant consequences if left undiagnosed.

The small, finger-shaped organ attached to the cecum, which is the beginning of the large intestine, is the appendix. Its function in the human body is minimal. But when the healthy cells in the appendix or the lining of the appendix mutate and grow out of control, they form a tumour.

How Appendix Cancer Develops?

Being a rare malignancy, appendix cancer frequently goes undetected until it has progressed or is unintentionally diagnosed during surgery for appendicitis. This form of malignancy develops when abnormal cells grow uncontrollably within the appendix, leading to the development of tumours. And, these cancers are called carcinoid tumours. The appendix can further rupture, which is typically how appendiceal cancer spreads to other cells and tissues of the abdomen, and also to other organs.

The exact cause of this mutation is still unclear, but certain factors, such as age, family history of gastrointestinal cancers, or genetic syndromes, can increase risk. The symptoms of most appendiceal cancers are similar to the symptoms of appendicitis, which include fever, leukocytosis, and acute abdominal pain in the right lower quadrant

Types of Appendix Cancer

Appendix cancer is classified into several types based on the kind of cells involved:

Carcinoid Tumours: The most common type, usually slow-growing, arising from neuroendocrine cells in the appendix lining.

Adenocarcinomas: Aggressive cancers that start in the glandular cells of the appendix and may resemble colon cancer in behaviour.

Mucinous Neoplasms: Tumours that produce mucus and can spread this material in the abdomen, potentially leading to a condition called pseudomyxoma peritonei (PMP).

Signet Ring Cell Adenocarcinomas: A rare and highly aggressive type that tends to spread rapidly.

Understanding the type of cancer is crucial because it influences both the staging and the choice of treatment.

Symptoms of Appendix Cancer

Appendix cancer often remains silent in its early stages because the appendix is small, and symptoms can mimic other gastrointestinal issues. When symptoms appear, they may include:

• Pain or discomfort in the lower right side of the abdomen
• Abdominal swelling or bloating
• Changes in bowel habits, such as diarrhoea or constipation
• Unexplained weight loss or loss of appetite
• Feeling full quickly after eating
• In women, abdominal or pelvic masses are mistaken for ovarian problems

In some cases, the first sign is acute appendicitis caused by a tumour blocking the appendix. Many diagnoses are made incidentally during surgery or imaging performed for other reasons.

Staging of Appendix Cancer

Once appendix cancer is diagnosed, determining its stage is essential. Staging helps doctors understand how far the disease has progressed and guides the treatment plan. The TNM staging system is commonly used:

• T (Tumour): How large the tumor is and how deeply it has invaded the appendix wall
• N (Nodes): Whether the cancer has spread to nearby lymph nodes
• M (Metastasis): Whether the cancer has reached distant organs

Stage 0 – Carcinoma in Situ

Cancer cells are present only in the innermost lining of the appendix. There is no spread to deeper tissues or other organs. Surgery to remove the appendix (appendectomy) is usually curative.

Stage I – Localized Disease

The tumour has grown deeper into the appendix wall but remains confined to the appendix. There is no lymph node or distant spread. Prognosis is generally favourable if the tumour is fully removed.

Stage II – Local Extension

Cancer has penetrated further, possibly into the fatty tissue around the appendix or nearby organs like the colon, but lymph nodes remain unaffected.

Stage II is subdivided into:

• Spread to the surrounding fatty tissue
• Growth through the appendix wall without organ involvement
• Direct extension to nearby organs without lymph node or distant spread

Stage III- Regional Lymph Node Involvement

The cancer has reached nearby lymph nodes. Sub-stages depend on how many lymph nodes are affected:

• 1–3 nodes involved
• Tumour growth plus 1–3 nodes affected
• 4 or more nodes involved

Treatment often requires a combination of surgery and chemotherapy.

Stage IV- Distant Metastatic Disease

• Cancer has spread beyond the local region. Sub-stages include:
• Spread to the abdominal lining (peritoneum) with well-differentiated tumour cells
• More widespread abdominal involvement with abnormal-looking tumour cells, possibly including lymph nodes
• Spread to distant organs outside the abdomen, such as the lungs

Why Staging Is Crucial?

Staging determines the treatment approach and prognosis. Early-stage cancers may be treated with surgery alone, while advanced stages often require aggressive therapy, including chemotherapy or participation in clinical trials for newer treatments. Accurate staging also provides clarity for patients and families, helping them understand the likely course of the disease and prepare for the next steps.

Appendix cancer may be rare, but its subtle onset and potential severity make awareness and timely detection essential. Recognizing the types, symptoms, and stages of the disease allows patients and caregivers to understand the seriousness of the diagnosis and the importance of tailored treatment. Early consultation with a healthcare professional and appropriate staging can significantly improve outcomes. With ongoing advancements in surgical techniques and oncology care, patients diagnosed at any stage have access to more effective and targeted treatment options than ever before.