The appendix is a pouch-like tube connected to the cecum, the first segment of the large intestine or colon. The appendix is around 10 centimeters (about 4 inches) long on average. It is a component of the gastrointestinal (GI) tract. The appendix, which is generally regarded to have no significant function in the body, may be a member of the lymphatic, exocrine, or endocrine systems.
Appendix cancer develops when healthy cells in the appendix mutate and proliferate uncontrollably. These cells combine to generate a tissue growth known as a tumor. A tumor might be malignant or noncancerous. A malignant tumor is one that can grow and spread to other regions of the body. Appendiceal carcinoma is another name for appendix cancer. A benign tumor is one that can develop but does not spread.
APPENDIX TUMOR TYPES
1. Neuroendocrine tumor: A neuroendocrine tumor begins in hormone-producing cells, which are found in minute amounts in practically every organ of the body. This is also known as a carcinoid tumor. A neuroendocrine tumor usually begins in the gastrointestinal system or the lungs, although it can also originate in the pancreas, testicles, or ovaries. Appendix neuroendocrine tumors are most commonly found at the appendix’s tip.
Neuroendocrine tumors account for approximately half of all appendix tumors. Appendix cancer normally does not cause symptoms until it has spread to other organs, and it frequently goes undiscovered until it is discovered through an examination or operation for another reason. A neuroendocrine tumor of the appendix that remains restricted to the site of origin has a high likelihood of being successfully treated surgically. Learn more about neuroendocrine tumors of the gastrointestinal tract.
2. Colonic-type adenocarcinoma: Colonic-type adenocarcinoma accounts for around 10% of appendix cancers and is typically seen at the appendix’s base. Appendicitis cancer appears and behaves similarly to the most frequent type of colorectal cancer. It frequently passes unrecognized, and the diagnosis is frequently diagnosed after or after appendicitis surgery. Appendicitis is an inflammation of the appendix that causes abdominal pain or swelling, loss of appetite, nausea, vomiting, constipation or diarrhea, inability to pass gas, and a low fever that appears after other symptoms.
3. Appendiceal mucoceles: Mucoceles are swellings or sacs caused by appendix wall bulge that are often filled with mucus. A mucocele can form as a result of a variety of benign to malignant diseases in the appendix. Mucinous cystadenomas and mucinous cystadenocarcinomas are two of these disorders. Mucinous cystadenomas, which are comparable to adenomatous polyps that can grow in the colon, are benign and do not spread. When they are found in the appendix, they can be fully removed surgically.
If the appendix ruptures, the cells may spread throughout the body and continue to leak a jelly-like substance called mucin into the belly. Mucin accumulation can cause abdominal pain, bloating, and abnormalities in bowel function, including bowel obstruction (blockage). Mucinous cystadenocarcinomas in the abdomen can have similar consequences to mucin, but they are malignant, which means they can spread to other regions of the body.
4. Paraganglioma: This is a rare tumor that arises from paraganglia cells, which are a collection of nerve tissue cells that persist in small deposits after fetal (pre-birth) development. Paraganglia is frequently present near the adrenal glands, as well as some blood arteries and nerves, particularly in the head and neck region of the body. This form of tumor is typically considered benign, and it is frequently effectively treated with surgical removal of the tumor.
5. Signet-ring cell adenocarcinoma: Signet-ring cell adenocarcinoma is an uncommon type of adenocarcinoma that is thought to be more aggressive and difficult to cure than other types of adenocarcinomas. Signet-ring cell adenocarcinoma is so named because the cell seems to have a signet ring inside it when viewed under a microscope. This sort of appendix cancer is likewise treated in the same way that colon cancer is.
6. Goblet cell carcinomas/Adenoneuroendocrines: Adenocarcinomas and neuroendocrine tumors share characteristics with goblet cell carcinomas (both described above). They are more aggressive than neuroendocrine tumors, and treatment is frequently comparable to adenocarcinoma treatment.
SYMPTOMS OF APPENDIX CANCER
Appendix cancer patients may have the following symptoms or indicators. Appendix cancer patients may or may not have any of these abnormalities. Alternatively, the origin of a symptom could be a medical disease other than cancer:
- Aches and pains in the abdomen or pelvic.
- Increased girth (waistline enlargement), with or without navel protrusion.
- Bowel function changes.
- Infertility.
- Appendicitis.
- Ascites (fluid buildup in the abdomen).
- Bloating.
Please consult your doctor if you are concerned about any changes you are experiencing. In addition to other questions, your doctor will inquire as to how long and how frequently you have been experiencing the symptom(s). This is done to assist in determining the cause of the condition, which is referred to as a diagnostic.
If cancer is discovered, symptom relief is an important element of cancer care and treatment. It is frequently initiated shortly after diagnosis and continues throughout treatment. This is known as palliative care or supportive care. Make an appointment with your doctor provider to discuss your symptoms, especially any new or changing symptoms.
STAGES AND GRADES OF APPENDIX CANCER
Staging describes where the cancer is present, whether or not it has spread, and whether or not it is impacting other sections of the body. Doctors utilize diagnostic tests to determine the stage of cancer, therefore staging may not be complete until all of the tests are completed. Knowing the stage assists the doctor in determining the best course of treatment and can help estimate a patient’s prognosis, or possibility of recovery. Distinct forms of cancer have different stage descriptions.
TNM system of staging
The TNM system is one technique that clinicians use to describe the stage. Doctors use diagnostic test and scan results to address the following questions:
- Tumor(T): What is the size of the main tumor? Where can I find it?
- Node(N): Has the cancer spread to your lymph nodes? If so, where are they and how many are there?
- Metastasis(M): Is the cancer in other parts of the body? If so, where and how much has it spread?
The results are aggregated to establish each person’s cancer stage. Depending on the type of cancer, there are five stages: stage 0 (zero) and stages I through IV (1 through 4). The stage provides a common language for doctors to describe the cancer so that they can collaborate to determine the best treatments.
Appendicitis cancer is staged in two ways by doctors. The first is for neuroendocrine tumors, while the second is for carcinomas, which include adenocarcinomas. More information on each component of the TNM system for appendix cancer may be found below:
STAGING: NEUROENDOCRINE TUMORS OF THE APPENNDIX
The TNM staging system is used to classify neuroendocrine tumors, as shown below:
Tumor (T)
The “T” plus a letter or number (0 to 4) is used in the TNM system to describe the size and location of the tumor. If the T is followed by a number in parenthesis or the letter “m,” it signifies the number of tumors discovered or the presence of multiple tumors. A T2(3), for example, indicates that the largest tumor identified is T2 in size and that there are three tumors. The size of a tumor is measured in millimeters (cm). A centimeter is approximately the width of a normal pen or pencil.
Stages can also be subdivided into smaller groups to assist define the tumor in greater depth.
Information about the tumor’s stage:
Stomach
TX: The cancer cannot be evaluated.
T0: There is no evidence of cancer.
T1: The tumor is 1 cm or less in size and has spread to a deeper layer of cells in the stomach, known as the lamina propria or submucosa.
T2: The tumor is more than 1 cm in size, or it has developed into the stomach’s muscle layer, known as the muscularis propria.
T3: The tumor has spread through the muscularis propria layer and into the subserosa layer underneath it.
T4: The tumor has migrated into surrounding organs or tissues, or it has grown into the layer of tissue on the outside of the stomach called the serosa or peritoneum.
Duodenum and ampulla of Vater (small intestine)
After the stomach, the duodenum is the first portion of the small intestine. The ampulla of Vater is a tiny bump in the duodenum where bile duct and pancreas enter the small intestine.
TX: The cancer cannot be evaluated.
T1: The tumor is 1 cm or smaller in size and only involves the top layer of mucus membrane or connective tissue on top of the duodenum’s muscle layer, or it only involves the sphincter of Oddi. The Oddi sphincter is a muscle that regulates the flow of digestive juices into the duodenum via the ampulla of Vater.
T2: The tumor is more than 1 cm in diameter. Or it has developed into the duodenum’s muscle layer, known as the muscularis propria, or through the sphincter of Oddi and into the duodenum.
T3: The tumor has spread to the pancreas or the tissue around it.
T4: The tumor has spread to the peritoneum or other internal organs.
Jejunum and ileum (small intestine)
The jejunum and ileum are tiny intestine segments that connect the duodenum to the large intestine.
TX: The cancer cannot be evaluated.
T0: There is no evidence of cancer.
T1: The tumor is one centimeter or less in size and only affects the top layers of tissue in the small intestine.
T2: The tumor is more than 1 cm in size or has spread into the small intestine’s muscle layer.
T3: The tumor has spread through the muscle layer and into the surrounding tissues, but it has not spread beyond the small intestine.
T4: The tumor has spread beyond the small intestine’s outer wall and into the peritoneum or other organs.
Appendix
TX: The cancer cannot be evaluated.
T0: There is no evidence of cancer.
T1: The tumor is no larger than 2 cm
T2: The tumor is larger than 2 cm but smaller than or equal to 4 cm.
T3: The tumor has developed into the layers of tissue on the outside of the appendix or is greater than 4 cm.
T4: The tumor has infiltrated adjacent organs or structures or has grown through the peritoneum.
Colon and rectum
TX: The cancer cannot be evaluated.
T0: There is no evidence of cancer.
T1: The tumor is 2 cm or smaller in size and has spread beyond the top layer of cells into the layers beneath, such as the lamina propria or submucosa.
- T1a: The tumor is less than one centimeter in size.
- T1b: The tumor is between one and two centimeter in size.
T2: The tumor has spread to the muscle (muscularis propria). Or it is larger than 2 cm and has infiltrated the lamina propria or submucosa.
T3: The tumor has migrated into the subserosal tissue behind the muscle.
T4: The tumor has infiltrated adjacent organs or structures or has expanded to the peritoneum.
Node (N) – for all sections of the gastrointestinal tract
The letter “N” in the TNM staging system denotes lymph nodes. These little, bean-shaped organs aid in the battle against infection. Regional lymph nodes are lymph nodes located near the site of the malignancy. Lymph nodes located in other sections of the body are referred to as distant lymph nodes.
NX: The lymph nodes are unable to be evaluated.
N0: The cancer has not spread to the lymph nodes in the surrounding area.
N1: The cancer has progressed to the lymph nodes in the surrounding area. Only the jejunum and ileum: The cancer has only progressed to 12 lymph nodes.
N2 (only the jejunum and ileum): The cancer has progressed to at least 12 lymph nodes and/or the tumors are greater than 2 cm in size.
Metastasis (M) – for all sections of the gastrointestinal tract
The “M” in the TNM system indicates if the cancer has migrated to other parts of the body, a condition known as metastasis.
M0: There is no lengthy spread.
M1: There is lengthy spread.
- M1a: The cancer has only progressed to the liver.
- M1b: Beyond the liver, the cancer has spread to at least one other organ, such as the lung, ovary, lymph node, peritoneum, or bone.
- M1c: The cancer has spread to both close and far-flung areas of the body.
Cancer stage grouping
Doctors integrate the T, N, and M information to determine the stage of the cancer.
Stomach
Stage I: There is a tiny tumor that has not spread to other parts of the body (T1, N0, M0).
Stage II: The tumor has grown larger and has reached the subserosa, which is located underneath the layer of muscle in the stomach. The malignancy has not spread to any lymph nodes or other parts of the body (T2 or T3, N0, M0).
Stage III: The tumor is of any size, and the cancer has spread to nearby lymph nodes (any T, N1, M0). Alternatively, the tumor has progressed to the outside of the stomach but not to the lymph nodes or elsewhere (T4, N0, M0).
Stage IV: There is distant metastasis (any T, any N, M1).
Duodenum and ampulla of Vater (small intestine)
Stage I: There is a tiny tumor that has not spread to other parts of the body (T1, N0, M0).
Stage II: The tumor is greater than 1 cm in diameter or has spread to the pancreas. The cancer has not spread to any lymph nodes or other parts of the body (T2 or T3, N0, M0).
Stage III: The tumor is of any size, and the cancer has spread to nearby lymph nodes (any T, N1, M0). Alternatively, the tumor has migrated to the peritoneum or other organs but has not expanded to the lymph nodes or elsewhere (T4, N0, M0).
Stage IV: There is distant metastasis (any T, any N, M1).
Jejunum and ileum (small intestine)
Stage I: There is a tiny tumor that has not spread to other parts of the body (T1, N0, M0).
Stage II: The tumor is more than 1 cm in diameter and has spread to the subserosa, which is located underneath the layer of muscle in the small intestine. The malignancy has not spread to any lymph nodes or other parts of the body (T2 or T3, N0, M0).
Stage III: The tumor is of any size, and the cancer has spread to nearby lymph nodes (any T, N1 or N2, M0). Alternatively, the tumor has spread to the peritoneum or other organs or tissues but has not migrated to the lymph nodes or elsewhere (T4, N0, M0).
Stage IV: There is distant metastasis (any T, any N, M1).
Appendix
Stage I: There is a tumor that is 2 cm or smaller in size and has not spread elsewhere (T1, N0, M0).
Stage II: The tumor has grown to more than 2 cm and has reached the membrane that joins the appendix to the abdominal wall. The cancer has not spread to any lymph nodes or other parts of the body (T2 or T3, N0, M0).
Stage III: The tumor is of any size, and the cancer has spread to nearby lymph nodes (any T, N1, M0). Alternatively, the tumor has progressed to the peritoneum or beyond but has not migrated to the lymph nodes or elsewhere (T4, N0, M0).
Stage IV: There is distant metastasis (any T, any N, M1).
Colon and rectum
Stage I: There is a tumor that is 2 cm or smaller in size and has not spread elsewhere (T1, N0, M0).
Stage IIA: The cancer has infiltrated the muscle. Or it is larger than 2 cm and has infiltrated the lamina propria or submucosa. There has been no spread of cancer (T2, N0, M0).
Stage IIB: The tumor has migrated into the subserosal tissue behind the muscle. The cancer has not spread to any lymph nodes or other parts of the body (T3, N0, M0).
Stage IIIA: The tumor has expanded to the peritoneum or has invaded surrounding organs or tissues, but it has not migrated to the lymph nodes or elsewhere (T4, N0, M0).
Stage IIIB: The tumor is of any size, and the cancer has spread to nearby lymph nodes (any T, N1, M0).
Stage IV: There is distant metastasis (any T, any N, M1).
Recurrent: Cancer that recurs after therapy is referred to as recurrent cancer. If the cancer returns, more tests will be performed to determine the degree of the recurrence. These tests and scans are frequently identical to those performed at the time of the first diagnosis. lymph nodes in the neck (any T, N1, M0).
Grade (G)
A GI tract NET may also be described by its grade (G), which ranges from 1 to 3. The grade of NETs defines how quickly the tumor cells grow and divide, a process known as mitosis.
The doctor counts the number of dividing cells visible in 10 high-power areas under a microscope to determine mitosis. This is known as the mitotic count. The Ki-67 index can also be measured by the doctor. Ki-67 is a protein that rises in cells as they prepare to divide. If there is a high percentage of Ki-67 positive cells in a region, it indicates that the cells are proliferating fast. The Ki-67 index measures the rate at which tumor cells proliferate.
The grade of the malignancy may aid the doctor in predicting how rapidly the NET may grow and spread. In general, the lower the grade of the tumor, the better the prognosis. The following grade scale applies to all forms of GI tract NETs:
GX: Grade cannot be evaluated.
G1: The mitotic count is less than two, or the Ki-67 index is less than three.
G2: The mitotic count ranges from 2 to 20, and the Ki-67 index ranges from 3 to 20.
G3: The mitotic count is greater than 20 or the Ki-67 index is greater than 20.
NETs in grades 1 and 2 tend to grow slowly. Grade 3 NETs grow quickly and are very aggressive. The final grade is determined by which of the two indices (mitotic count or Ki-67) places the tumor in the highest grade category.
Differentiation degree
Examining cells under a microscope can also reveal the degree of differentiation. The degree to which a cancer cell resembles a healthy cell is referred to as differentiation.
Tumors that have been well-differentiated appear more like healthy cells and develop more slowly. Tumors that are well-differentiated can be graded 1, 2, or 3; the well-differentiated grade 3 category is new to the World Health Organization pathology classification.
Poorly differentiated cells resemble healthy cells less and grow faster. Poorly differentiated tumors are always grade 3 and are classified as neuroendocrine carcinomas (NECs) with large-cell and small-cell subtypes.
STAGING: CARCINOMAS OF THE APPENDIX
Tumor (T)
TX: The primary tumor is unable be evaluated.
T0: In the appendix, there is no sign of cancer.
Tis: This is called carcinoma in situ (also called cancer in situ). Cancer cells are exclusively found in the first layers of the appendix’s lining.
Tis (LAMN): A low-grade appendiceal mucinous neoplasm (LAMN) restricted by the muscularis propria, the appendix wall’s muscle layer. Mucin in the absence of cells may infiltrate the muscularis propria.
T1: The tumor has infiltrated the submucosa, the appendix’s innermost layer.
T2: The tumor has infiltrated the muscle propria.
T3: The tumor has developed past the muscularis propria and into the appendix’s subserosa (a thin layer of connective tissue) or into the mesoappendix, which is a fatty tissue area adjacent to the appendix that gives the appendix with blood supply.
T4: The tumor has spread through the visceral peritoneum, the lining of the abdominal cavity, or it has infiltrated other surrounding organs.
T4a: The tumor has spread throughout the visceral peritoneum.
T4b: Other organs or structures, such as the colon or rectum, have been invaded by the tumor.
Node (N)
NX: Due to a lack of information, the regional lymph nodes cannot be evaluated.
N0: There is no evidence of regional lymph node metastasis.
N1: The cancer has progressed to one to three regional lymph nodes.
N2: The cancer has spread to at least four regional lymph nodes.
Metastasis (M)
M0: The cancer has not spread.
M1: There is distant metastasis.
M1a: This is an area of spread known as intraperitoneal acellular mucin.
M1b: This term refers to a metastasis within the peritoneum that extends beyond M1a.
M1c: This is a metastasis that has spread beyond the peritoneum.
Grade (G)
GX: The grade of the tumor cannot be determined.
G1: Tumor cells have a high level of differentiation.
G2: The tumor cells have a moderate level of differentiation.
G3: Tumor cells are not well differentiated.
G4: Tumor cells are not differentiated.
Carcinomas of the appendix cancer stage grouping
Stage 0: This refers to cancer in situ. The cancer has only been identified in one location and has not spread (Tis, Tis(LAMN), N0, M0).
Stage I: The cancer has spread to the inner layers of appendix tissue, but not to the regional lymph nodes or other regions of the body (T1 or T2, N0, M0).
Stage IIA: The cancer has spread to the connective or fatty tissue next to the appendix but not to the regional lymph nodes or other sections of the body (T3, N0, M0).
Stage IIB: The cancer has expanded through the appendix lining but has not gone to the regional lymph nodes or other parts of the body (T4a, N0, M0).
Stage IIC: The tumor has spread to other organs, such as the colon or rectum, but not to the regional lymph nodes or other sections of the body (T4b, N0, M0).
Stage IIIA: The cancer has expanded to the inner layers of appendix tissue as well as one to three regional lymph nodes, but it has not moved to other regions of the body (T1 or T2, N1, M0).
Stage IIIB: The cancer has expanded to neighboring appendix tissue or via the appendix lining, as well as 1 to 3 regional lymph nodes, but has not moved to other parts of the body (T3 or T4, N1, M0).
Stage IIIC: This stage denotes cancer that has spread to four or more regional lymph nodes but has not migrated to other parts of the body (any T, N2, M0).
Stage IVA: Stage IVA refers to any of the following scenarios:
- This stage refers to cancer that has progressed to other parts of the abdomen but not to the regional lymph nodes (any T, N0, M1a).
- This stage denotes a cancer that has migrated to other sections of the abdomen and may have expanded to the regional lymph nodes; the cancer cells are well differentiated (any T, any N, M1b, G1).
Stage IVB: The cancer has migrated to other parts of the abdomen and may have expanded to the regional lymph nodes; the cells are moderately or poorly differentiated (any T, any N, M1b; G2, G3, or GX).
Stage IVC: The cancer has migrated from the abdomen to other regions of the body, including the lungs (any T, any N, M1c, any G).
Recurrent: Recurrent cancer is cancer that has returned after therapy for both neuroendocrine tumors and carcinomas. If the cancer returns, more tests will be performed to determine the degree of the recurrence. These tests and scans are frequently identical to those performed at the time of the first diagnosis.
DIAGNOSIS OF APPENDIX CANCER
Many tests are used by doctors to detect or diagnose cancer. They also perform tests to see whether the cancer has spread to another place of the body from where it began. This is referred as as metastasis. Imaging tests, for example, can reveal whether or not the cancer has spread. Images of the inside of the body are produced via imaging tests. Doctors may also conduct tests to determine which treatments are most likely to be effective.
A biopsy is the only guaranteed way for a doctor to know if a part of the body has cancer in most cases of cancer. During a biopsy, the doctor extracts a small sample of tissue for laboratory testing. If a biopsy is not possible, the doctor may recommend alternative tests to aid in the diagnosis.
Not all of the tests listed will be administered to every individual. When selecting a diagnostic test, your doctor may take the following variables into account:
1. The type of cancer that is suspected.
2. Your symptoms and signs.
3. Your age and general health.
4. The outcomes of previous medical tests.
The following tests, in addition to a physical exam, may be performed to identify appendix cancer:
1. Biopsy: A biopsy is the removal of a small sample of tissue for microscopic examination. Other tests can indicate the presence of cancer, but only a biopsy can provide a definitive diagnosis. The material is next examined by a pathologist (s). A pathologist is a medical professional who specializes in interpreting laboratory tests and assessing cells, tissues, and organs to identify disease.
However, appendix cancer is most typically discovered unexpectedly after or after abdominal surgery for another reason. If cancer is detected during surgery, the doctor will take a section of the colon and surrounding tissue (referred to as a margin) for evaluation. An appendectomy, or surgical removal of the appendix, is frequently performed on a patient. This is typically done for what is initially assumed to be appendicitis, and the cancer is found after the pathologist processes and reviews the tissue under the microscope.
Depending on the type of appendix cancer (such as a neuroendocrine or adenocarcinoma tumor) and the size of the tumor, further operation may be necessary to remove another margin of tissue around the place where the tumor formed (if it is a neuroendocrine tumor). Appendix cancer can sometimes be identified by chance during a CT scan for another cause.
2. Magnetic response imagery (MRI): An MRI produces detailed images of the body by using magnetic fields rather than x-rays. The tumor’s size can be determined via an MRI. To provide a crisper image, a special dye known as a contrast medium is administered before to the scan. This dye can be injected into a patient’s vein or given to them in the form of a pill to ingest.
3. Computed tomography (CT) scan: A CT scan uses x-rays captured from various angles to create images of the inside of the body. A computer combines these images to create a detailed, three-dimensional (3D) image that identifies any anomalies or malignancies. A CT scan can be performed to determine the size of the tumor. To improve image detail, a specific dye known as a contrast medium is sometimes administered before to the scan. This dye can be injected into a patient’s vein or given to them in the form of a pill or liquid to consume.
4. Ultrasound: An ultrasound creates a picture of the interior organs by using sound waves.
5. Radionuclide scanning: These tests are only used to detect neuroendocrine tumors, not other types of appendix cancer. In a vein, a small amount of a radioactive, hormone-like material that is attracted to a neuroendocrine tumor is injected. After that, a specialized camera is utilized to show where the radioactive chemical collects. This method can be used to detect the spread of a neuroendocrine tumor.
After the diagnostic tests are completed, your doctor will go through the results with you. If appendix cancer is the diagnosis, these data will also assist the doctor in describing the cancer. This is referred to as staging.
APPENDIX CANCER TREATMENTS
Different types of specialists frequently collaborate in cancer care to develop a patient’s overall treatment plan, which mixes many sorts of therapy. This is referred to as a multidisciplinary team. Other health care professionals on cancer care teams include physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, nutritionists, and others.
The type and stage of cancer, potential side effects, and the patient’s preferences and overall health all influence treatment options and recommendations. Treatment for symptoms and side effects, which is an important element of cancer care, may also be part of your treatment plan. Take the time to read about all of your treatment options, and don’t be afraid to ask clarifying questions.
Discuss the aims of each treatment with your doctor, as well as what you can expect during treatment. These discussions are known as “shared decision making.” When you and your doctors collaborate to choose therapies that meet the goals of your care, this is referred to as shared decision making. Because there are various treatment choices for appendix cancer, shared decision making is especially crucial.
Surgery
During an operation, the tumor and some surrounding healthy tissue are removed. It is the most commonly used method of treating appendix cancer. Appendix cancer is typically low-grade (see Stages and Grades) and thus slow-growing. It is frequently treatable alone by surgery. A surgical oncologist is a specialist who specializes in the surgical treatment of cancer.
Appendix cancer surgeries include the following:
- Appendectomy: The surgical removal of the appendix is known as an appendectomy. An appendectomy is not always the procedure used to treat this type of cancer. Instead, it is more common for people to have an appendectomy for appendicitis, and later cancer is diagnosed when the tissue is sent to a pathologist. If the pathology report identifies a neuroendocrine tumor that is smaller than 1 or 2 cm in size, an appendectomy may be sufficient to eliminate the cancer. However, if the neuroendocrine tumor is larger or has aggressive characteristics, or if it is not a neuroendocrine tumor, such as an adenocarcinoma or goblet cell carcinoma, a second operation to remove more tissue will most likely be required.
- Debulking surgery: Debulking (or cytoreduction) surgery for later-stage appendix cancer may be recommended depending on the kind of appendix cancer and if there is just dissemination in the abdominal cavity. The doctor eliminates as much of the tumor “bulk” as feasible during this operation, which can assist the patient even if it does not remove every cancer cell from the body. Debulking surgery is sometimes followed by chemotherapy (see below) to kill any leftover cancer cells.
- When a tumor produces mucous, the majority of the aberrant tissue is typically not malignancy but the result of mucus accumulation. Because the mucus seems to resemble jelly, this condition is also referred to as “jelly belly.” Removing mucus from the gut can typically ease bloating in patients.
- Hemicolectomy: A hemicolectomy may be advised for neuroendocrine tumors greater than 2 cm in size or non-neuroendocrine appendix malignancies. This procedure involves the excision of a section of the colon near the appendix. Often, neighboring blood arteries and lymph nodes are removed at the same time. A right hemicolectomy is surgery performed on the colon’s right side. Despite the removal of a major portion of the large intestine, the procedure normally does not need the use of a colostomy or stoma, which is a hole in the abdomen through which the bowel contents are evacuated into a bag.
- Removal of the peritoneum: Some surgeons advocate aggressive surgery to remove the peritoneum (abdominal lining) in order to remove as much malignancy as feasible. This procedure is also known as a peritonectomy. Not all surgeons feel that significant surgery, such as a peritonectomy, is required in patients with slow-growing, low-grade appendix cancer that has progressed beyond the colon to other parts of the abdomen. Peritonectomy can be useful in eliminating the bulk of cancer cells in patients with a very slow-growing tumor. Even if it does not eradicate every cancer cell, this can benefit the patient by reducing the amount of malignancy. However, it is a tough procedure with serious adverse effects. Before proposing this extensive surgery, the doctor will consider a variety of criteria, including the patient’s age and overall health. Patients should consult with a professional who specializes in this type of procedure before undergoing the procedure.
Before undergoing surgery, consult with your health care team about the potential adverse effects of the procedure.
Radiation therapy
The use of high-energy x-rays or other particles to eliminate cancer cells is known as radiation therapy. A radiation oncologist is a doctor who specializes in the use of radiation therapy to treat cancer. A radiation therapy regimen, or schedule, typically consists of a predetermined number of treatments administered over a predetermined time period.
Appendix cancer is rarely treated with radiation therapy. When the cancer has spread to a specific location, such as the bone, it may be utilized to ease symptoms.
Radiation therapy might cause fatigue, moderate skin responses, upset stomach, and loose bowel motions. The majority of negative effects fade quickly after treatment is completed.
Chemotherapy
Chemotherapy is the use of medications to eradicate cancer cells, typically by preventing the cancer cells from growing, dividing, and proliferating.
Chemotherapy is most commonly given shortly after surgery for appendix cancer that is not a neuroendocrine tumor when cancer is diagnosed outside the appendix region. Depending on the stage of disease, it may also be given after surgery for non-neuroendocrine appendix cancer to prevent the cancer from returning.
Chemotherapy is classified into several forms based on how the chemicals are given to the body:
- Local or intraperitoneal chemotherapy: The medicine is administered to a single location or section of the body during local chemotherapy. This is known as intraperitoneal chemotherapy, which is chemotherapy administered straight into the abdominal cavity. Typically, the surgeon will aim to remove as much of the tumor as possible (see above for more information on debulking surgery) and then install a tube in the abdomen via which chemotherapy can be administered following the operation. In some situations, the chemotherapy is heated above body temperature to improve its capacity to permeate tissue that may be lined with tumor cells; this is referred to as hyperthermic (or heated) intraperitoneal chemotherapy (referred to as HIPEC). When the treatment is finished, the tube is usually withdrawn without the need for extra surgery.
- Systemic chemotherapy: This type of chemotherapy is administered into the bloodstream in order to reach cancer cells located throughout the body. This can be accomplished using an intravenous (IV) tube, which is a tube that is inserted into a vein with a needle, or with a pill or capsule that is taken (orally). Some people may receive this sort of chemotherapy at their doctor’s office or an outpatient clinic, while others may be admitted to the hospital.
A chemotherapy regimen, or schedule, typically consists of a predetermined number of cycles administered over a predetermined time period. A patient may be administered one medicine at a time or a mixture of drugs at the same time.
Systemic chemotherapy for non-neuroendocrine appendix malignancies is comparable to that used for colorectal cancer. Fluorouracil (5-FU), leucovorin (folinic acid), capecitabine (Xeloda), irinotecan (Camptosar), and oxaliplatin are examples of chemotherapy drugs (Eloxatin).
Chemotherapy side effects vary depending on the individual and the dose used, but they can include exhaustion, infection risk, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These adverse effects normally fade away once the treatment is completed.
Targeted therapy
Targeted therapy is a type of cancer treatment that targets specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This method of treatment inhibits cancer cell growth and spread while limiting damage to healthy cells.
The targets of all cancers are not the same. Your doctor may order tests to determine the genes, proteins, and other variables in your tumor in order to find the most effective treatment. This enables clinicians to provide the most effective treatment to each patient whenever possible. Furthermore, research studies are continuing to learn more about specific molecular targets and new treatments aimed at them.
Non-neuroendocrine appendix cancer is treated with targeted therapies similar to colorectal cancer, such as bevacizumab (Avastin), ziv-aflibercept (Zaltrap), ramucirumab (Cyramza), cetuximab (Erbitux), and panitumumab (Vectibix).
Discuss with your doctor the potential side effects of a certain medicine and how to manage them.
REMISSION AND THE POSSIBILITY OF REOCCURENCE
When cancer cannot be identified in the body and there are no symptoms, the patient is said to be in remission. This is often known as having “no evidence of illness,” or NED.
Remission can be either temporary or permanent. Many people are concerned that the cancer will recur as a result of this uncertainty. While many remissions are durable, it is vital to discuss the potential of the cancer returning with your doctor. Understanding your recurrence risk and treatment options may make you feel more prepared if the cancer returns.
Recurrent cancer occurs when the cancer returns after the initial treatment. It may return in the same location (called a local recurrence), nearby (called a regional recurrence), or elsewhere (distant recurrence).
When this happens, a fresh round of testing begins to discover as much as possible about the recurrence. Following the completion of this testing, you and your doctor will discuss treatment choices. Surgery, chemotherapy, targeted therapy, and radiation therapy are frequently included in treatment plans, but they may be done in a different mix or at a different pace. Your doctor may recommend that you participate in clinical studies that are looking for new ways to treat this sort of recurring cancer. Palliative care will be essential for reducing symptoms and side effects regardless of the treatment plan you choose.
People suffering from recurring cancer frequently experience emotions such as bewilderment or anxiety. You are urged to discuss these feelings with your health care provider and inquire about support options to assist you in coping.
CONCLUSION
Cancer recovery is not always achievable. If treatment fails, the condition may be referred to as advanced or terminal cancer.
This is a traumatic diagnosis, and many people find it difficult to address advanced cancer. However, it is critical to communicate openly and honestly with your health care team in order to express your views, preferences, and concerns. The health care team has specialized skills, expertise, and information to assist patients and their families. It is critical to ensure that a person is physically comfortable, pain-free, and emotionally supportive.
People with advanced cancer who are projected to live for less than six months may wish to consider hospice care. Hospice care is intended to offer patients nearing the end of their lives with the highest possible quality of life. You and your family are invited to discuss hospice care alternatives with the health care team, which may include hospice care at home, a designated hospice center, or other health care venues. Staying at home with nursing care and proper equipment might be a viable choice for many families.
