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Appendix Cancer: Types, Symptoms, Causes & Treatment

Appendix Cancer

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Overview

Appendix cancer, also recognized as appendiceal cancer, is a form of cancer that is rarely found. It occurs when the cells in your appendix change (mutate), and they grow out of control.

Concisely, appendiceal cancer-forming cells grow and multiply out of control. Cancerous epithelial cells develop in the appendix itself.

The small adjoining appendage of your digestive system called the appendix is situated lower down the right side of your abdomen and at the junction of your large and small intestines. However, scientists do not know what your appendix is for, but some believe it helps support your immune system. Others, however, argue the possibility that it is just a vestigial organ - one that is no longer expressed to any significant degree.

Appendix Cancer: Types, Symptoms, Causes & Treatment

Types of Appendix Cancer

There are various benign (noncancerous) tumors and malignant tumors that may arise in your appendix. Malignant would mean they can grow and spread. Malignant appendix tumors may be further categorized depending on the type of cell that gives origin to them. Types include:

  • Appendiceal adenocarcinoma
  • Neuroendocrine Tumors

Appendiceal Adenocarcinoma (Epithelial Tumors)

Most appendix cancers are appendiceal adenocarcinomas. These develop from gland-like tissue lining the appendix. They have several classes:

  • Mucinous adenocarcinoma: This starts on the lining of your appendix, releasing mucin, a component of mucus. These tumors can spread, but most do not spread farther than the abdomen.
  • Colonic-type (nonmucinous) adenocarcinoma: These tumors develop at the base of your appendix. They behave like colon cancer tumors and cause many of the same symptoms.
  • Signet ring cell adenocarcinoma: This rare type may sometimes arise from the appendix, with mucin secretion and storage by a high amount of cancer cells. It is called signet ring adenocarcinoma because of its ring-like appearance under a microscope.
  • Goblet cell adenocarcinoma: This very rare adenocarcinoma shows some neuroendocrine features.

Appendiceal Neuroendocrine Tumors

Cancer of the neuroendocrine system primarily targets neuroendocrine cells that respond to nervous system stimulation and affect the release of hormones. They are typically slow-growing tumors.

How many appendix cancers are there?

Appendix cancer is very rare. It is estimated to affect 1 to 2 persons per million per year in the United States.

Risk Factors

Other factors associated with an increased risk of appendix cancer include:

  • Age:

    Although it can manifest at any age, it is most commonly diagnosed in people aged 50s.

  • Sex:

    Females tend to develop neuroendocrine tumors in their appendix.

  • Smoking or tobacco use:

    Tobacco-related activities may increase the risk of all cancers, including appendix carcinoma.

  • Medical history:

    Appendiceal carcinoma may be associated with specific conditions including atrophic gastritis, pernicious anemia, and Zollinger-Ellison syndrome.

  • Family history of cancer:

    Some studies suggest that people with appendix cancer may inherit genes that increase their risk, though more research is needed.

Symptoms

It is possible to have appendix cancer without any symptoms arising at all. Alternatively, they might be felt at an advanced stage when the tumor has spread in extent. Here are common symptoms associated with appendix cancer,

  • Appendicitis (inflammation or infection of your appendix).
  • Bloating (fullness or tightness in your belly).
  • Fluid buildup in your abdomen (ascites).
  • Increase in waist size.
  • Pain in your abdomen or pelvis.
  • Changes in bowel habits (including diarrhea).
  • Nausea and vomiting.
  • Feeling full soon after you start eating (early satiety).

Most of the time, the appendix cancer is paired with a very rare phenomenon called pseudomyxoma peritonei (PMP). In PMP, the cancerous cells secrete jelly-like mucin which makes the appendix swell up. In a longer duration, symptoms appear, such as a bloated stomach as well as abdominal pain.

Appendix Cancer Symptoms and Causes Image
Appendix Cancer Symptoms and Causes Image

Causes

Appendix cancer starts when the cells in your appendices grow and mutate into out-of-control forms. However, it is still unknown to the experts as to why the processes of mutation and uncontrolled growth are initiated.

Diagnosis

Diagnosing colon cancer involves a series of tests and procedures that help doctors confirm if cancer is present, understand how far it may have spread, and decide on the best treatment plan. These tests also help rule out other conditions that might cause similar symptoms. Below are the most common methods used to detect colon cancer.

01
Appendix Cancer Diagnosis CT and MRI Image
CT and MRI
Such imaging modalities as CT and MRI translate into very detailed internal pictures and possibly help identify cancerous tumors or evidence of its spreading, especially in nearby abdominal organs or into the peritoneum.
02
Appendix Cancer Diagnosis Laparoscopy Image
Laparoscopy
allows a provider to visualize real time of the appendix and nearby associated tissue with an instrument called a laparoscope passed through a small incision in the abdomen into the cavity.
03
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Biopsies
Most commonly, biopsies identify cancers as they observe tissue under a microscope. Since the biopsy of the appendix directly is very difficult, the doctor may take a sample from other sites where it might have spread.
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Blood Tests
Blood tests can give pointers for diagnosis and prognosis, especially after a cancer diagnosis has been made. These tests measure the proteins that indicate how well the disease has traveled and progressed.

Staging Appendix Cancer

The next step on the road to an effective treatment plan and understanding of what to expect will be staging the cancer in the appendix. Staging varies widely based on the tumor type concerned:

  • Doctors stage appendiceal adenocarcinomas using the TNM system (T=tumor size, N=lymph node involvement, and M=spread elsewhere). A stage from I to IV is assigned based on these factors, with I being early stage and IV being late stage.
  • For appendiceal neuroendocrine tumors of the appendix, staging depends on whether the tumor can be completely respected and its grade or whether it looks more abnormal from a histological point of view. Low-grade tumors have better prognosis than high-grade for cases that are completely respectable.

The stage of appendix cancer is complicated and very difficult. This is owing to multiple forms of tumors, each responding differently to the treatment, and it has specific behavior too. Therefore, we would strongly recommend that you discuss with your provider the implications of this tumor type and stage for your care and hence prognosis either for yourself or someone else.

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Treatments

Surgery and drug treatment are the common options that objective medical attention employs in the treatment of the appendix cancer patient. Different criteria like tumor size and stage, extent of spread, patients overall health, and most importantly, personal treatment desires define a particular treatment plan for each patient.

Surgical Treatment

Usually the first line of treatment, surgery against appendix cancer tends to vary depending on the further advanced stage of the tumor:

Appendix Cancer Treatment Appendectomy Image
Appendectomy
For small and early tumors (generally no larger than 1-2 cm), the simple excision of the appendix is likely to suffice as surgery.
Appendix Cancer Treatment Hemicolectomy Image
Hemicolectomy
More aggressive or bigger tumors may require a far more aggressive form of treatment referred to as hemicolectomy. This involves the removal of part of the colon with some of the adjacent lymph nodes and the appendix.
Appendix Cancer Cytoreductive Surgery Image
Cytoreductive Surgery
Cytoreductive surgery is carried out to rid the abdominal cavity of cancer to the maximum possible extent with the debulking procedure involved, sometimes including parts of one or more affected organs.

Medication-Based Treatments

Drug treatment is usually done in instances where surgery is not sufficiently effective, and cancer metastasizes. In such cases, drug treatment is offered to control the disease and improve the outcome:

Appendix Cancer Chemotherapy Image
Chemotherapy
This can be defined as standard treatment with strong drugs to destroy cancerous nature cells which have either spread beyond the appendix or remained after surgery. It can be given mainly orally and through other routes by injections.
Appendix Cancer HIPEC Treatment Image
HIPEC Treatment
This is where HIPEC treatment comes in, a specially devised highly aggressive treatment. HIPEC is a form of chemotherapy delivered during surgery directly into the abdominal cavity, wherein using heated chemotherapy drugs with greater efficacy against tumor cells and less toxicity is expected.
Appendix Cancer Targeted Drug Therapy Image
Targeted Drug Therapy
These are newer-targeted effects that attach cancer cells to healthy tissue and are designed toward very specific genes or proteins that drive tumor cell growth. One generally used drug for this kind of therapy concerning appendix cancer is either called cetuximab, bevacizumab, ramucirumab, or panitumumab.

Side Effects of Treatment

A variety of side effects may be observed after treatment for appendiceal cancer, such as surgery, chemotherapy, or targeted drug therapy; depending on the type of treatment, the specific drug combinations used, and the patient's individual bodily response, these side effects may vary.

The following are some of the more common short-term and long-term side effects that accompany surgical procedures, such as appendectomy, hemicolectomy, or cytoreductive surgery:

  • Pain and Discomfort: Usually, there will be pain, swelling, and discomfort in the area after surgery. Pain can be managed reasonably well by medication.
  • Risk of Infection: As with any surgery, there can always be infections, either at the site or within.
  • Digestive Difficulties: More radical surgery, such as colectomy and removal of associated organs, can slow bowel movement, change defecation habits, and lead to malabsorption.
  • Fatigue and Weakness: A feeling of weakness or tiredness may persist for a few weeks after the surgery during recovery.

Chemotherapy kills rapidly dividing cells, including noncancerous ones in the GIT, bone marrow, and hair follicles. The most common side effects are:

  • Nausea and Vomiting: Probably the most common side effects; treated with medications controlling nausea.
  • Hair Loss: Hair loss varies from partial to complete, depending on the regimen, and is almost always temporary.
  • Fatigue: Most patients feel tired during the course of chemotherapy as it affects the whole body.
  • Increased Infection Risk: Chemotherapy lowers white blood cell counts, leading to immunosuppression.
  • Mouth Sores and Diarrhea: Inflammation of the digestive tract can cause soreness and changes in bowel habits.

There could be special side effects associated with hot abdominal chemotherapy (HIPEC), performed during surgery.

  • Abdominal Pain and Swelling: Heat and direct drug exposure can cause discomfort and bloating.
  • Disruption of Digestion: Temporary changes in digestion such as nausea, slowed bowel function, or constipation are common.
  • Infection and Fluid Imbalance: This post-procedure follow-up is crucial in minimizing these risks.

Most likely, targeting therapies would be very specific to cancer cells, but side effects are expected:

  • Skin Reactions: Rashes, dry skin, or sensitive skin are common side effects associated with medications such as cetuximab or panitumumab.
  • Hypertension: Some targeted therapies like bevacizumab may increase blood pressure.
  • Fatigue and Diarrhea: This is the most common symptom but usually not as severe as in conventional chemotherapy.
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Support

Since fighting appendix cancer can be a major struggle for its victims, emotionally and mentally, as well as financially, it is then important to know what resources are available for either the patient or the family supporting someone through this difficult time.

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Counseling and therapy

Counseling and therapy provide a safe and rending place to articulate ones fears, anxieties, and grieving with the help of a licensed therapist or counselor. Most cancer centers will have psychological support services to assist both patients and caregivers.
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Support Groups

Support groups, those formed in the physical world or online, allow the experience of sharing one's stories, tips, and encouragement with all of these others, thereby fostering community life.
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Nutrition Support

With the impact of cancer treatment on appetite and digestion, registered dietitians help to prepare an individualized nutrition plan aimed at optimal treatment and energy needs.
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Financial Aid Programs

Medical expenses, medications, and other services may receive assistance through organizations such as the Cancer Financial Assistance Coalition (CFAC), HealthWell Foundation, and Patient Advocate Foundation.
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Spiritual Support

Many patients find comfort talking with chaplains, spiritual advisors, or faith-based counselors. Most hospitals have spiritual care teams who aid people regardless of their beliefs.

Research

Appendix cancers are rare, but the popularity of this field keeps increasing. With advances being made in genetic studies, novel drug therapies, and surgical approaches, things are promising for better treatment outcomes and life quality. If you affect or know of anyone affected, connecting with specialized cancer centers along with research foundations may be useful in accessing newer treatments and support.

Prevention Image

Prevention

These precautions may reduce your chances of developing any kind of cancer from the appendix, even if there is medically no formula for prevention:

Eat well, exercise regularly, avoid smoking, and limit alcohol.

Keep an eye out for potential symptoms, such as abdominal pain and bloating, and seek medical consultation as soon as possible.

If any members of your family have a history of cancer, consider getting genetic counseling and starting annual screening tests.

Be sure tissue appendices are tested for the presence of cancer once they have been surgically removed.
Questions to Ask your Healthcare Provider about Appendix Cancer

Getting education about the condition at stake can prove useful in making informed decisions regarding the health benefits. Some questions for your provider include:

  • Q1. Which type of appendix tumor have I got?
  • Q2. How big or hefty is the tumor?
  • Q3. What is the stage and grade of appendix cancer I have?
  • Q4. What would be the treatment options available for me?
  • Q5. When do I need to start therapy to cure this?
  • Q6. Will I be able to continue going to work treating this cancer?
  • Q7. Are any resource or support groups available to me?
Questions to Ask your Healthcare Provider about Appendix Cancer Image