What does it mean?

5-FU is a chemotherapy drug which can be used to treat the symptoms appendix cancer. It is also used to treat cancer of the colon, breast, stomach, and pancreas. It is also used in a cream to treat certain skin conditions.
5-FU stops cells from making DNA and it may kill cancer cells. It is a type of antimetabolite. Also called 5-fluorouracil and fluorouracil.

An adenoma is a type of tumour. An adenoma of the appendix grows within the appendix and then breaks out through the appendix wall. The appendix may reseal afterwards. Whilst the adenoma itself may be benign, the condition is still fatal is left untreated.

The definition of an adenoma is a benign tumour of epithelial tissue with glandular origin, glandular characteristics, or both. Although not common, this type of tumour can become malignant.

Cannot spread to distant parts of the body.

Related to mucin production and generally associated with ovarian cancer and gastrointestinal cancers, CA-125 is a protein often found on the surface of ovarian cancer cells and in some normal tissues.

It is used as a marker for ovarian cancer as well as for PMP. However, CA -25 levels may also be high in other types of non-cancerous conditions, including menstruation, pregnancy and pelvic inflammatory disease.

Cancer antigen 19-9 (CA 19-9) is a protein that exists on the surface of certain cells. CA 19-9 does not cause cancer; rather, it is a protein that is produced by the tumour cells, making it useful as a tumour marker to follow the course of the cancer.

CA 19-9 is elevated in most patients with pseudomyxoma peritonei and advanced pancreatic cancer, but may also be elevated in other cancers and diseases such as bowel cancer, lung cancer and gall bladder cancer, as well as in benign diseases such as gall stones, pancreatitis, cystic fibrosis, and liver disease. Because elevated levels of CA 19-9 are seen in many diseases, elevated levels do not necessary mean the presence of pancreatic cancer.  Very small amounts of CA19-9 may also be found in healthy patients.

Cancer is a disease that occurs when the body’s cells start to grow and multiply uncontrollably, leading to the formation of abnormal masses of tissue called tumours. These tumours can be benign, meaning they are not cancerous and do not spread to other parts of the body, or they can be malignant, meaning they are cancerous and can spread to other parts of the body through the bloodstream or lymphatic system. Pseudomyxoma peritonei (PMP) behaves differently.

A carcinoma is cancer that begins in a tissue that lines the inner or outer surfaces of the body.

Associated with the spread of tumour in the body cavity  CEA is a protein that is normally not detected in the blood of a healthy person. When certain types of cancer are present, CEA may be produced by the cancer cells. CEA may then be detected in blood, but it will not indicate which kind of cancer is present.

CEA is often used to monitor patients with cancers of the gastrointestinal (GI) tract such as bowel (colorectal) cancer. It may be raised in other cancers, such as ovarian and breast cancers, but can also be raised in benign conditions such as liver disease and inflammatory bowel disease (Crohns disease or ulcerative colitis).
It can also be high in heavy smokers and for other reasons.

Chemotherapy is a type of cancer treatment using drugs to kill cancer cells and/or limit their growth. For appendix cancer patients, there are a number of ways that chemotherapy can be given. In addition to eh way that these drugs are usually administered to the patient — by slow infusion into a vein and as tablets orally — appendix cancer and PMP patients often receive heated intraperitoneal chemotherapy (HIPEC) and/or EPIC (intraperitoneal chemotherapy).

This is the complete surgical tumour removal. This is a long operation which takes about 10 hours to complete and includes:

• removal of the right hemicolon
• spleen
• gall bladder
• greater omentum and lesser omentum
• stripping of the peritoneum from the pelvis and diaphragms
• tripping of tumour from the surface of the liver
• removal of the uterus and ovaries in women
• removal of the rectum in some cases

This is also referred to as a full peritonectomy.  The goal is to remove tumour nodules down to 2.5mm.

A CT scan is used for diagnostic purposes. It’s a form of radiography in which your organs are scanned with X-rays and the results are put together by a computer to generate images of parts of the body. PMP patients usually have a chest, abdomen and pelvis CT.

Instructions you will be given for your CT scan will depend on where you have it done – the sensitivity of the scanning machines is different so what you need to do beforehand and what will happen on the day will vary.

Formerly disseminated peritoneal adenomucinosis

The least-aggressive and least-invasive type of appendix cancer which it has spread (disseminated) throughout the abdomen (peritoneal cavity). These cancerous cells divide quickly and so do not to respond to systemic chemo.  There is usually lots of mucin (musinosis) with adenomucinosis. This term is generally outdated.

Dysplasia is a description of cells that look abnormal under the microscope but are not cancerous.

Endoscopy is a procedure where a doctor puts a tube-like instrument into the body to look inside it. There
are many types of endoscopy, each of which is designed for looking at a certain part of the body. PMP patients will most frequently have a a colonoscopy (a look at the inside of the colon) or a sigmoidoscopy (a look at the sigmoid colon, the bit of your colon before your rectum and anus).

Early Postoperative Intraperitoneal Chemotherapy

As part of the Sugarbaker techinque, EPIC is the administration of a chemotherary drug directly into the abdomen for a number of days (say 3 or 4) after surgery.  It is also used to refer to the administration of chemotherapy directly into the abdomen following surgery as an alternative to HIPEC.

Fluoropyrimidine is one of a group of substances used to treat cancer. A fluoropyrimidine is a type of antimetabolite – antimetabolites can have toxic effects on cells, such as halting cell growth and cell division.
Fluoropyrimidines used to treat appendix cancer and pseudomxoma peritonei include are capecitabine, floxuridine, and fluorouracil (5-FU).

The name goblet cell tumour refers to the shape of the cells as they appear under a microscope – they are shaped like wine goblets.

High-grade appendiceal mucinous neoplasm.

Hyperthermic Intraperitoneal Chemotherapy

Heated chemotherapy, delivered directly into the abdomen, after complete cytoreduction.  If the surgeons are able to remove the all the tumours, you will be given a heated chemotherapy drug.  This is usually Mitomycin C. This is put directly in your abdomen while you are in theatre and the chemotherapy is left in for 90 minutes.  HIPEC will penetrate tumour nodules up to 2.5mm in size.  The heat increases cytotoxicity of chemotherapy

Chemotherapy given into the abdomen during surgery is called intraoperative chemotherapy.

Hyperthermic Intrathoracic Chemotherapy (HITOC) is a specialized medical procedure that involves delivering chemotherapy drugs directly to the thorax (chest) while the patient is under general anesthesia. The drugs are heated to a temperature of around 42°C, which is thought to enhance their effictiveness in killing cancer cells.

Intravenous (IV) means into or within a vein. Intravenous usually refers to giving a drug or other substance through a needle or tube inserted into a vein.

Low-grade appendiceal mucinous neoplasm.

Anything abnormal on or in the body, not just neoplasms.

Lynch syndrome is an inherited disorder in which affected individuals have a higher-than-normal chance of developing colorectal cancer and certain other types of cancer, often before the age of 50. It can also be called hereditary nonpolyposis colon cancer or HNPCC.

Can spread to distant parts of the body.

high-grade mucinous carcinoma peritonei

Moderately or poorly differentiated adenocarcinomas

low-grade mucinous carcinoma peritonei

Low grade mucin producing disease including appendiceal adenoma, well-differentiated mucinous carcinomatosis, well-differentiated variants of mucinous adenocarcinomas.

How quickly cells are dividing. Tumours with fewer dividing cells are usually low grade.

“Mother of all Surgeries“

This is a nickname for the big cytoreductive surgery – full peritonectomy and HIPEC. It was thought up by the wife of an appendix cancer patient some years ago on her blog.

No Evidence of Disease

This is the term used when there are no signs of cancer in your body at a given time.

Nasogastric tube

A narrow tube which is passed through the bose into the stomach.  It is usually then taped to your nose.  It can be used to aspirate (to remove fluid) from your stomach after surgery or to administer nutition directly to the stomach (enteral nutrition).

Peritoneal Mesothelioma (also referred to as malignant peritoneal mesothelioma, peritoneal malignant mesothelioma, malignant primary peritoneal mesothelioma and abdominal mesothelioma) is a rare, usually aggressive, form of cancer affecting the serosal (inner) surface of the peritoneum.

Peritonectomy is the surgical removal of areas of the peritoneum.

The peritoneum is the tissue that lines your abdominal wall and covers most of the organs in your abdomen.

PIPAC stands for Pressure-Controlled Intermittent Positive-Pressure Aerosol Chemotherapy. It is a specialized medical procedure that involves delivering chemotherapy directly to the abdominal cavity using a combination of pressure and aerosol.

Low-dose Pressurized Intrathoracic Aerosol Chemotherapy (PITAC) is a specialized medical procedure that involves delivering low doses of chemotherapy drugs directly to the thorax (chest) using a pressurized aerosol system. The purpose of PITAC is to deliver the chemotherapy drugs directly to the cancer cells in the chest, while minimizing exposure of other parts of the body to the drugs.

A polyp is a growth that protrude from a mucous membrane.
When polyps are attached to the mucous membrane by a thin stalk they are called pendunculated polyps; if no stalk is present, they are sessile polyps.

Pseudomyxoma peritonei or PMP

“Pseudomyxoma peritonei” literally means “false mucinous tumour in the abdomen”. In the 1860s, the first mucinous tumours were discovered in the chest, surrounding the heart. Doctors called them “myxomas.” When a surgeon found a mucinous tumour in the abdomen twenty years later, he called it a “pseudomyxoma” (false myxoma), because the chest tumours were discovered first.

This phrase is sometimes used to refer to all types of appendix cancer.  It is sometimes used to refer to the mucin only, or to the less-aggressive form of appendix cancer.

Recurrence is used to describe cancer that has come back, usually after a period of time during which the cancer or disease was not present or could not be detected (no evidence of disease).  This can also be called recurrent
cancer or disease.

The name signet cell tumours comes from the appearance of the cells under a microscope; signet ring cells resemble signet rings.

Small Bowel Obstruction

A small bowel obstruction is an obstruction of the small bowel which means the small bowel effectively becomes blocked.  There can be a number of causes for this with the main one being adhesions following surgery.

The Sugarbaker technique was developed by Paul Sugarbaker at the Washington Cancer Institute. It involves complete surgical tumour removal combined with intraoperative heated chemotherapy, followed by postoperative intraperitoneal chemotherapy.

Total Parenteral Nutrition

Parenteral Nutrition is the administration of nutrients intravenously.  When this is the only source of nutrition, it is called Total Parenteral Nutrition or TPN.  It is often given after surgery to allow the intestines to rest and contains a balance of carbohydrates, fats, minerals and vitamins.

Enteral nutrition is the delivery of balanced nutrition directly usually into the stomach but also to the duodenum.

A mass due to autonomous growth of cells, whether benign or malignant.

The visceral peritoneum is the layers of tissue that cover the outer surface of most organs in the abdomen, including the intestines.

A “WOC” (Wound, Ostomy and Continence) nurse, previously known as an “ET” (Enterostomal Therapy) nurse, is often the best medical professional for dealing with problems involving ostomies. These nurses are also often known as stoma care nurses or, more simply, stoma nurses.