About Appendix Cancer
Appendix Cancer is quite a rare condition which affects around 2 in a million people according to research data. Majority of cases of Appendix Cancer are diagnosed incidentally during an appendectomy done to treat appendicitis. What role does the appendix plays in the overall health is not clearly understood but many physicians believe that it contributes to the overall immune health of an individual. Because of the rarity of the condition very limited data is available but it is believed to cause rupture of the appendix and this is the first presentation of Appendix Cancer [1].
Majority of the cases of appendiceal tumors are cancers to the tune of 70-80%. The remaining 20% are adenosarcoma or lymphosarcoma. An individual with Appendix Cancer will have acute abdominal pain localized to the right lower quadra`nt. This will be accompanied by fever at times. Appendectomy is the frontline treatment for Appendix Cancer. In case if the condition is diagnosed while performing another abdominal procedure, then appendectomy is performed concurrently to get the tumor out [1].
In majority of the cases, the tumors are small and the surgery is good enough to treat Appendix Cancer. However, in rare instances the tumor size is large enough to indicate additional treatments in the form of chemotherapy and repeat procedures. Coming to the anatomy of the appendix, it is a tubular sac which connects to the colon. The function of this organ is not fully understood as people who have had their appendix removed remain perfectly healthy with no concerns whatsoever. This article highlights the presenting features and the treatment options available for Appendix Cancer [1].
What Are The Symptoms And Treatment Options For Appendix Cancer?
Appendix Cancer remains asymptomatic for the most part in the initial stages. It is only when the cancer has advanced that it is able to be diagnosed as that is when it starts to cause certain symptoms. However, by this time, the cancer in all probability has spread to the nearby organs. In some cases, however, a physician may find a tumor incidentally when examining a patient for an entirely different reason [2].
An individual with Appendix Cancer will experience:
Pseudomyxoma Peritonei: This is a condition that occurs when there is a rupture of the appendix. This causes the tumor cells to infiltrate into the abdominal cavity. The cancer cells produce a protein called mucin that starts to accumulate in the abdominal cavity and starts to spread rapidly. If the condition remains untreated then it may lead to intestinal blockages or problems with the digestive system of the affected individual [2].
The symptoms of Pseudomyxoma peritonei include waxing and waning abdominal pain along with visible enlargement or swelling of the abdomen. The patient will also have early satiety and significant loss of appetite. There may also be diarrhea alternating with constipation [2].
Appendicitis: This is yet another presenting feature of Appendix Cancer. It is believed to be the one of the first presenting features of this condition. This condition occurs when there is an inflammation of the appendix. The cause for this inflammation is that the tumors block the passage of the appendix and the bacteria that are present within it get trapped and start to grow. This condition requires emergent medical attention which is usually surgery [2].
The procedure to treat appendicitis is named appendectomy where the appendix is removed in its entirety. After removal of the appendix a biopsy of the tumor will be done and the diagnosis of Appendix Cancer can be confirmed. The primary symptoms of appendicitis include severe abdominal pain localized to the area between the belly button and the right lower quadrant. This pain tends to get worse with any attempts at movement or activity that requires bending or lifting [2].
The onset of pain is sudden with rapid worsening. There may also be swelling of the abdominal region along with periods of nausea and vomiting. It should be noted here that not all Appendix Cancers cause appendicitis. This is because some form of cancers has tumors that develop at the tip of the appendix and this does not result in any inflammation as there is no blockage caused [2].
The reverse of it is also true in that it is not necessary for anyone with appendicitis to have Appendix Cancer. Appendicitis can also be caused due to other gastrointestinal disorders like inflammatory bowel disease [2].
Some of the other signs that may indicate an Appendix Cancer are the presence of a lump on the abdominal or pelvic region which can be easily palpated by the individual. In females, there have been many cases where Appendix Cancer has been mistaken for an ovarian cancer [2].
In case of a malignant Appendix Cancer, the cancer may spread to nearby organs and infiltrate the lining of the abdominal cavity. It is medically referred to as peritoneal carcinomatosis. If not treated on time, it may lead to intestinal blockage with its inherent complications. The usual areas where a malignant Appendix Cancer can spread to are the liver, spleen, uterus, and the ovaries. In majority of the cases, Appendix Cancer does not go beyond the abdominal cavity [2].
The treatment of Appendix Cancer is determined keeping in mind the type of the tumor, presence of any metastasis, and the overall health status of the patient. If the cancer is fairly localized to the appendix then removing the appendix suffices as a treatment option. If the cancer has spread to other organs then more extensive surgery may have to be done to remove the parts that have been damaged by the cancer cells. This may include the ovaries or parts of the intestines [2].
According to the American Association of Endocrine Surgeons, majority of people benefit greatly from appendectomy along with removal of a part of the colon on the right side especially in cases of a large tumor. This procedure is called right hemicolectomy. In some cases, physicians may opt to do chemotherapy in addition to the surgery to get rid of any remaining cancer cells especially in cases where the cancer has spread [2].
A recent development in chemotherapy treatment specifically for Appendix Cancer has gained preference. This procedure is called heated intraperitoneal chemotherapy or HIPEC. This is extremely beneficial for malignant Appendix Cancer that has spread to the abdominal cavity. This procedure involves filling the abdominal cavity with chemotherapy solution and allowing it to take its effect. The medication kills every cancer cell present within the abdominal cavity [2]. This is a relatively new treatment and has a prolonged recovery time of up to a couple of months or even more. However, many researchers believe that this is perhaps the best treatment for people with Appendix Cancer to have the best outcome possible [2].
The prognosis of an individual with Appendix Cancer depends on the type of tumor and how much the cancer has spread. The 5 year survival rate for people with Appendix Cancer where the tumor is less than 3 cm in its dimensions and has not spread is almost 100%. This goes down to around 80% if the cancer has spread to involve the lymph nodes [2].
The 5 year survival rate remains at close to 80% if the tumor size is larger than 3 cm irrespective of whether it has spread or not. The survival rate however plummets down to 30% of there is significant metastasis and other vital organs of the body become involved [2].
In conclusion, Appendix Cancer is an extremely rare condition where a tumor develops in the appendix. The prevalence of this condition is around 2 in a million which speak for the rarity of this condition. This condition is completely asymptomatic during the initial stages and is either incidentally diagnosed or is diagnosed when it has spread to other parts and starts causing symptoms. People who have Appendix Cancer can be treated by removing the appendix although in some cases physicians may also do chemotherapy in addition to the surgery for removal of the appendix [1, 2].
Many factors with regard to the etiology and pathogenesis of this condition remain unclear due to the rarity of the condition and not much data being available. However, people with Appendix Cancer may get help from support groups where they can come in contact with other patients with this condition or patients who have been successfully treated [1, 2].
Appendix Cancer is definitely treatable and the 5 year survival rate is quite good in majority of the cases. However, once diagnosed it is essential to start treatment for this condition immediately to prevent it from spreading and affecting the overall prognosis of the individual [2].
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