Appendix cancer treatment

Appendix cancer is a rare type of cancer in which the healthy cells in the appendix become abnormal and start growing rapidly. These cancerous cells become a tumor or mass inside the appendix. 

The appendix is a tube that appears like a pouch or small sack. The appendix is connected to the colon (the longest part of the large intestine) at the beginning of the large intestine. 

There is no known function of the appendix. It is believed to play a role in the immune (disease-fighting) system of the body. However, people can live a healthy and normal life without an appendix.

The different types of appendix cancer include:

1. Neuroendocrine tumor or carcinoid tumor: It usually starts at the tip of the appendix and is the cause for more than half of the appendiceal malignancies (cancers).
2. Mucinous cystadenoma or low-grade mucinous neoplasm: It is a benign tumor starting in the mucoceles, which are sacs filled with mucus in the appendix wall. It is usually a benign (non-cancerous) type of appendix tumor.
3. Mucinous cystadenocarcinoma: This type of appendix tumor starts in the mucoceles and is of the malignant (cancerous) type.
4. Colonic-type adenocarcinoma: This type of appendix tumor starts at the appendix base.
5. Goblet cell carcinoma or adenoneuroendocrine tumor: This type of tumor is more aggressive than a neuroendocrine tumor and may spread to the other organs. It has characteristics similar to a neuroendocrine tumor and an adenocarcinoma.
6. Paraganglioma: This is a rare type of benign appendix tumor.
7. Signet-ring cell adenocarcinoma: This is a rare, malignant type of tumor that is difficult to treat.

The exact cause of appendix cancer is not known. The appendix tumors are formed when the cells undergo abnormal changes and multiply at a quick rate. The excess cells can build up and lead to the formation of a tumor. This tumor can spread to the different organs and tissues of the body. The cause of abnormal cellular development is unknown.

Certain factors increase the chances of developing appendix cancer. These factors include:

• Age between 40 and 60 years
• Smoking
• Family history of multiple endocrine neoplasia type 1 or MEN1 (a disorder leading to the formation of tumors in hormone-producing glands)
• Pernicious anemia (vitamin B-12 deficiency)
• Atrophic gastritis (long-term inflammation of the lining of the stomach)
• Zollinger-Ellison syndrome (a digestive system disorder in which the tumors lead to the production of too much acid in the stomach)

The symptoms of appendix cancer may not be seen initially. The symptoms may become noticeable during an imaging test, surgery, or any other medical procedure. The symptoms may include:

• Bloated stomach (abdomen)
• Severe or chronic (long-term) abdominal pain
• Obstruction of the bowel 
• Diarrhea
• Hernia (bulging of a tissue or organ through an abdominal opening)
• Discomfort in the lower right abdominal region
• Ovarian masses (the growths that may occur on or in the ovaries, the reproductive organs of women)

Appendix cancer usually shows no symptoms initially. It is usually diagnosed by the doctor when the tumor has spread to the other organs leading to various symptoms, or accidentally during an imaging test, or after an appendicitis surgery (removal of the appendix due to inflammation). The following diagnostic tests may be performed:

1. Physical examination: The doctor will examine you physically, and check the symptoms. The doctor will also note down any medical history or family history of MEN1.
2. Imaging tests: Imaging tests like CT scan, MRI scan, Ultrasound helps in obtaining clear images of the internal organs of the body. This helps in checking if there is any tumor visible in and around the appendix.
3. Colonoscopy: A colonoscope is a long tube with a camera on one end. It is inserted in the rectum and moved up to the colon to check for the presence of any tumors.
4. Biopsy: In this procedure, a small sample of the tissue growth is excised (removed) by the doctor and sent to the laboratory to check for the presence of any abnormal or cancerous cells.

The treatment of appendix cancer depends on the type of tumor present, if cancer has spread to the other parts of the body, and if the person has any pre-existing medical conditions.

The various forms of treatment for appendix cancer include:
1. Surgery:
a) Appendectomy: If the cancer is localized to the appendix only, removal of the appendix is the best form of treatment. This is known as appendectomy.
b) Hemicolectomy: In some cases of appendix cancer, or when the tumor is larger in size, the doctor may recommend the removal of one-half of the colon and some lymph nodes. The surgical procedure done to remove half of the colon is known as hemicolectomy.
c) Cytoreductive surgery/debulking: If cancer has spread to the other parts of the body, the surgeon removes the tumor, the surrounding fluid, and sometimes the nearby organs attached to the tumor.

2. Chemotherapy:
Chemotherapy is a form of treatment in which certain drugs or groups of drugs may be used to treat cancer.
In the case of appendix cancer, chemotherapy may be required before or after the surgery.
Chemotherapy is usually performed if the tumor is larger than 2 centimeters, cancer has spread to the surrounding areas and lymph nodes, or the cancer is very aggressive.
The different types of chemotherapy include:

a) Systemic chemotherapy, which may be given in the form of pills to be taken orally, or injected into the vein (intravenously).
b) Regional chemotherapy, which is given directly into the abdomen, like intraperitoneal chemotherapy (chemotherapy drugs are put inside the abdomen directly) or hyperthermic intraperitoneal chemotherapy (filling the abdominal cavity with chemotherapy drugs that have been heated).
c) A combination of regional and systemic chemotherapy
The doctor will follow up on your treatment with periodic imaging tests like CT scans or MRI scans to check if the tumor has been completely eliminated.

The side effects associated with appendix cancer treatment may include:

• Ascites (fluid buildup in the abdominal cavity)
• Pain
• Nausea
• Vomiting
• Constipation or diarrhea
• Hair loss
• Loss of appetite
• Bleeding
• Infection

The complications associated with appendix cancer may include:

• Obstruction of the bowel
• Blood clot (a clump of blood that changes from the liquid state to a gel-like semi-solid state)
• Pain in the abdomen

Contact your doctor if you have any of the above symptoms.

Although appendix cancer cannot be prevented as such, a few lifestyle changes may lower your risk of developing appendix cancer:

• Quit smoking
• Drink alcohol in moderation
• Eat a healthy, well-balanced diet
• Maintain your weight
• Exercise regularly

The 5-year survival rates for appendix cancer after the removal of the appendix according to a 2011 review are as follows:

• 94 percent, if the carcinoid tumor is confined to only the appendix
• 85 percent, in case the appendix cancer has spread to the nearby areas or lymph nodes
• 34 percent, in case the appendix cancer has spread to some distant organs (rarely seen in carcinoid tumors)

The survival rate improves in cases of appendix cancers where a part of the colon is also removed and chemotherapy has been performed.

The prognosis and outlook for most people having early-stage appendix cancer are generally good. 
Most of the time, appendix cancer remains undetected until an imaging test or appendix surgery is being performed for some other medical reason. 
It is important to have regular follow-up appointments with your doctor after the diagnosis of appendix cancer to check if there is any recurrence of cancer. 

The cost of appendix cancer treatment in India varies across different cities and hospitals.