Cancer

What is Hemochromatosis and what are its causes?

Hemochromatosis (HE-mo-kro-ma-TO-sis) is a disease in which too much of iron builds up in your body (iron overload).

Overview
- Iron is a mineral found in many foods.
- Too much iron is toxic to your body.
- It can poison your organs and cause organ failure.
- In hemochromatosis, iron can build up in most of your body’s organs.
- It builds especially in the liver, heart, and pancreas.
- Too much iron in the heart can cause irregular heartbeats called arrhythmias (ah-RITH-me-ahs) and heart failure.
- Too much iron in the pancreas can lead to diabetes.
- If hemochromatosis isn’t treated, it may even cause death.

Too much iron in the liver can cause:
- an enlarged liver,
- liver failure,
- liver cancer,
- cirrhosis (sir-RO-sis): scarring of the liver, which causes the organ to not work well.

Types of Hemochromatosis
The two types of hemochromatosis are primary and secondary.
- Primary hemochromatosis is caused by a defect in the genes. These genes control how much iron you absorb from food.
- Secondary hemochromatosis usually is the result of another disease or condition that causes iron overload.

The basic two types of hemochromatosis are:
- Hereditary hemochromatosis: Hemochromatosis is mainly associated with a defect in a gene called the HFE gene.
- Acquired hemochromatosis: It occurs as a result of certain medical conditions.

Primary Hemochromatosis
- It is inherited from their parents.
- If you inherit two hemochromatosis genes—one from each parent—you’re at risk for iron overload.
- The two faulty genes cause your body to absorb more iron than usual from the foods you eat.
- The severity of hemochromatosis also varies.

Certain factors that can affect the severity of the disease are:
- A high intake of vitamin C can make hemochromatosis worse.
- This is because vitamin C helps your body absorb iron from food.
- Alcohol use can worsen liver damage.
- Cirrhosis caused by hemochromatosis.
- Conditions such as hepatitis also can further damage or weaken the liver.

Hereditary Hemochromatosis
- It is caused by a mutation in a gene that controls the amount of iron your body absorbs.
- The mutations that cause hereditary hemochromatosis are passed from parents to children.

Gene mutations that cause hemochromatosis:
- The gene that is mutated most often in people with hereditary hemochromatosis is called HFE.
- You inherit one HFE gene from each of your parents.
- If both parents pass mutated HFE genes to you, you may develop hemochromatosis.
- The HFE gene has two common mutations, C282Y and H63D.
- Genetic testing can reveal whether you have these mutations in your HFE gene.

If you inherit two abnormal genes:
- One may develop hemochromatosis.
- Not everyone with two abnormal genes develops signs and symptoms of hemochromatosis.
- You can also pass the mutation on to your children.

If you inherit one abnormal gene:
- One won’t develop hemochromatosis.
- But your body may absorb more iron than normal.
- You are considered a gene mutation carrier and can pass the mutation on to your children.

How hemochromatosis affects your organs?
- Patients with hereditary hemochromatosis may absorb as much as 30 percent of the iron that is ingested.
- Your body can’t use or eliminate this extra iron, it’s stored in the tissues of major organs, especially the liver.
- Eventually you may accumulate five to 20 times as much iron as normal.
- Over a period of years, the stored iron can severely damage many organs, leading to organ failure and chronic diseases such as cirrhosis and diabetes.

Other types of hemochromatosis

Juvenile hemochromatosis
- This causes the same problems in young people that hereditary hemochromatosis causes in adults.
- Iron accumulation begins much earlier and symptoms usually appear between the ages of 15 and 30.
- Although juvenile hemochromatosis is an inherited disease, the genetic abnormalities that cause it don’t involve the HFE gene.
- It is caused by a mutation in a gene called hemojuvelin.

Neonatal Hemochromatosis
- In this severe disorder, iron builds up rapidly in a baby’s liver and can cause death.

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What are different treatment options available for treating liver cancer?

These are some of the treatments available:
- Surgery,
- Immunotherapy,
- Photodynamic Therapy,
- Hyperthermia,
- Radiation Therapy
- Radiosurgery
The best option for curing liver cancer is surgery.

Other techniques used to treat liver cancer are:
- Including inserting needles into the tumor.
- Destroying the tumor (ablation).
- Injecting a substance into the tumor to deprive it of the blood supply it needs (embolization).
- Chemotherapy
- Radiation therapy

The best treatment for the liver cancer is based on:
- Age, overall health, and medical history.
- Extent of the disease.
- Stage of the cancer.
- Your tolerance of specific medicines, procedures, or therapies.
- Expectations for the course of the disease.
- Your opinion or preference.

Surgery
- Surgery is the only way to cure liver cancer.
- The most common type of surgery for liver cancer is resection (removal of the cancer).
- The presence of cirrhosis of the liver makes surgical resection less successful.
- It may require the entire liver to be removed and replaced with a donated liver (liver transplant).
- Surgery depends on whether the part of your liver that is not affected by the cancer is healthy.
- When part of your liver is removed, enough healthy liver tissue left is to be taken care of, to carry out all of the critical jobs of the liver.

Ablation
- Ablation destroys the tumor without removing it.
- It is a good option for patients with small liver tumors.
- This process uses high-energy radio waves, alcohol injections and very cold metal probes to destroy the tumor.

Embolization
- Tumors need the oxygen supplied by blood to grow.
- Embolization stops blood from flowing to the tumor.
- Embolization can be done by injecting substances that plug the artery.
- This is sometimes combined with chemotherapy (chemoembolization) or radiation therapy (radioembolization).

Chemotherapy
- Chemotherapy drugs kill cancerous cells.
- It works by stopping cancer cells from growing or reproducing, which kills the cells.
- Chemotherapy may be taken by mouth in pill form or injected into a vein or muscle.
- Sometimes it is injected into a hepatic artery through a thin tube (catheter).
- This process is known as chemoembolization.

Radiation Therapy
- Radiation therapy uses high-energy radiation to kill cancer cells and shrink tumors.
- Radiation therapy for liver cancer is usually delivered internally.
- It is done by inserting a radioactive substance into the body.

Targeted Therapy
- Targeted therapy blocks the steps involved in the growth and proliferation of cancer cells.
- Targeted therapy drug may be taken by mouth or in a pill form.
- The main targeted therapy for liver cancer is a drug called Nexavar (sorafenib tosylate).

Hepatocellular Carcinoma
- Partial hepatectomy to resect the entire tumor.
- Liver transplantation.
- Cryoablation.
- Chemoembolization.
- Radiotherapy.
- Sorafenib.
- Radiofrequency ablation.
- Radiofrequency ablation combined with local chemotherapy.

Cholangiocarcinoma
- Photodynamic therapy.
- Brachytherapy.
- Radiotherapy.
- Liver transplantation.

Hepatoblastoma
- Chemotherapy, including vincristine, cyclophosphamide, and doxorubicin.
- Radiotherapy.
- Liver transplantation.
- Surgical resection.

How is liver cancer diagnosed?

Liver cancer is also known as hepatocellular carcinoma, HCC. It is a cancer that originates in liver. Liver cancers are malignant tumors that grow on the surface or inside the liver.

Diagnosis of Liver Cancer

Blood Tests
- Liver cancer depends so much on the vigilance of the physician screening with a tumor marker (alpha-fetoprotein) in the blood and radiological imaging studies.
- If the blood tests become abnormal or worsen due to liver cancer, this usually signifies extensive cancerous involvement of the liver.
- in liver cancer, the cancerous liver cells may take on the characteristics of other types of cells.
- For example, liver cancer cells sometimes can produce hormones that are ordinarily produced in other body systems.
- These hormones then can cause certain abnormal blood tests
- These include a high red blood count (erythrocytosis), low blood sugar (hypoglycemia) and high blood calcium (hypercalcemia).
- High serum cholesterol (hypercholesterolemia), is seen in up to 10% of patients from Africa with liver cancer.
- The high cholesterol occurs because the liver cancer cells are not able to turn off (inhibit) their production of cholesterol.
- There is no reliable or accurate screening blood test for liver cancer.
- The most widely used biochemical blood test is alpha-fetoprotein (AFP)
- AFP is a protein normally made by the immature liver cells in the fetus.

In adults, high blood levels (over 500 nanograms/milliliter) of AFP are seen in only three situations:
- Liver cancer.
- Germ cell tumors (cancer of the testes and ovaries).
- Metastatic cancer in the liver (originating in other organs).

There are a number of other liver cancer tumor markers like:
- Des-gamma-carboxyprothrombin (DCP),
- A variant of the gamma-glutamyltransferase enzymes,
- Variants of other enzymes (for example, alpha-L-fucosidase).

Imaging Studies
These studies provide information
- as to the size of the tumor.
- the number of tumors.
- whether the tumor has involved major blood vessels locally or spread outside of the liver.

Ultrasound Examination
- It is usually the first study ordered if liver cancer is suspected in a patient.
- The accuracy of an ultrasound depends very much on the technician and radiologist.
- Computerized axial tomography (CT scan) is a very common study used in the U.S.
- The ideal CT study is a multi-phase, spiral CT scan using oral and intravenous contrast material.

Pictures are taken in three phases:
- Without intravenous contrast.
- With intravenous contrast (enhanced imaging) that highlights the arterial system (arterial phase).
- When the contrast is in the venous phase.