Appendix Cancer: An Overview

Introduction

Appendix cancer is a very rare cancer derived from an appendix cell. With unobtrusive manifestations and symptoms, often it evades detection. The appendix is a thin, finger-like pouch attached to the large intestine; though it is not really very big, if such cancer develops in this organ, it can lead to really serious complications in one’s health if not found and treated on time.

Types of Appendix Cancer 

There are many different types of appendix cancer. Each of them has its very unique features, and some are discussed below:

Carcinoid Tumors:

These are the most common type and present from hormone-producing cells within the appendix. Cancerous tumors are usually slow growing. 

Adenocarcinomas:

These arise from glandular cells, as with colon cancer. From both the cellular structure and behavior, they are often much more aggressive than carcinoid.

Mucinous Adenocarcinoma:

Produces a large amount of mucus that invades the abdominal cavity and may lead to pseudomyxoma peritonei (PMP), a condition characterized by the thick accumulation of mucus within the belly. 

Goblet Cell Carcinoid:

Combination of patterns of carcinoma cell types present in the carcinoid and adenocarcinoma types. These tumors are aggressive and can only be diagnosed in advanced stages.

Signet-Ring Cell Adenocarcinoma:

It is a rare, aggressive type with poor prognosis. Its prognosis is poorer compared to other appendix cancer types. 

Causes and risk factors 

Appendix:

Cancer causes unknown, but these are the identified risk factors. 

Genetic Mutations:

Particular gene mutations contribute to the development of appendix cancer, although hereditary factors are far less clearly defined than they are in most other types of cancers. 

Family History:

A family history of gastrointestinal cancers may increase the risk; however, it is not a very common risk. 

Age and gender:

Patients are diagnosed with appendix cancer between 40 and 60 years; however, several kinds of appendix cancer more often affect women.

Appendix:

Cancer Symptoms This type of cancer most usually tends to present with very non-specific symptoms, almost like appendicitis, a condition that the patient may be suffering from. These symptoms are mostly: 
Abdominal Pain: It is usually on the lower right abdomen side, and it may be intermitting or continuous.

Swelling and Bloating:

This seems to appear more obvious in such cases where mucinous adenocarcinoma has filled the abdomen with mucus.

Changes in Bowel Habits:

Constipation or diarrhea may occur, sometimes alternating.

Unexplained Weight Loss:

Significant weight loss without any changes in diet or exercise can be a sign of many cancers, including appendix cancer. 

Digestive Problems:

Nausea, vomiting, and an overall feeling of discomfort in the abdomen can occur. 
Because these symptoms are nonspecific, many appendix cancers are detected incidentally during other abdominal surgeries or image tests.

Stages of Appendix Cancer 

The stages of appendix cancer are categorized by size, spread, and involvement in the lymph nodes:

Stage 0 (Carcinoma in Situ):

Carcinomas in situ are present; however, these neoplastic cells are restricted to the innermost lining of the appendix.

Stage I:

The tumor is confined within the appendix and exhibits neither lymph node spread nor spread to other organs.

Stage II:

Cancer begins to metastasize beyond the epithelium layer of the wall of the appendix and invades tissues neighboring the appendix, if present. 

Stage III:

Cancer may involve regional lymph nodes but remains within the confines of the peritoneal cavity. 

Stage IV:

Cancer spreads to other sites in the body or disseminates throughout the peritoneal cavity, such as the liver or lungs. 

Diagnosis 

Appendiceal cancer is difficult to diagnose because it is rare, and symptoms are often very nonspecific. Nevertheless, a diagnosis can sometimes be made based upon some of the following:

Imaging Tests:

Techniques, like CT scans, MRIs, and ultrasounds, may determine where the tumor lies and help understand its scope. 

Blood Tests:

Although not specific for appendix cancer, a marker such as CEA would give one an idea of the progress of the disease. 

Biopsy:

A tissue sample from the appendix is examined to ascertain the type and stage of the cancer.

Exploratory Surgery:

If the diagnosis is not very clear, exploratory surgery may be utilized in order to aid in viewing inside the abdomen as well as to obtain tissue samples directly for examination.

Treatment Options 

Appendix:

Cancer Treatment depends on the type, size, and stage of the tumor, as well as the overall health of the patient. 

Surgery:

This is considered the initial line of treatment when the cancers are restricted to the tumor. Surgery can range from a simple appendectomy, in which only the appendix is removed, to a more extensive procedure if cancer has extended into other parts. 

Cytoreductive Surgery with HIPEC (Hyperthermic Intraperitoneal Chemotherapy):

For mucinous types that involve the spreading in the abdominal cavity, HIPEC is cytoreduction surgery, followed by heated chemotherapy solution directly administered in the abdominal cavity, aiming at getting rid of any remaining cancerous cells left after the surgery.

Chemotherapy:

In advanced-stage patients, systemic chemotherapy may be given as a form of therapy for controlling symptoms and slowing down further disease progression. 

Targeted Therapy:

Targeted therapy is less common, and patients with cancer are administered only on a few conditions, more specifically for advanced cancers that prove resistant to the traditional cures.

Prognosis and Outlook

Appendix cancer has a totally different prognosis regarding different types and stages. Carcinoids, for example, are usually favorable if taken early, whereas signet-ring cell adenocarcinomas have a poor prognosis because they tend to aggressively invade body tissues and therefore follow poor prognosis. Early detection, therefore, will translate into high chances of survival, and this is only achieved with regular follow-ups and attention to unusual symptoms, especially for those at risk.

Conclusion 

Although appendix cancer is such an infrequent disease, if not treated on time, it can cause severe complications. Recognizing its symptoms, risk factors, and diagnosis methods is essential for early detection. Advances in treatment of this rare condition, such as HIPEC, have led to much better forecasting in a lot of cases.