Paraganglioma

Paraganglioma (or extra-adrenal pheochromocytoma) is a rare type of neuroendocrine tumour that develops outside of the adrenal glands, which sit on top of the kidneys. These types of tumours develop from a specific type of chromaffin cell called paraganglia cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be cancer cells
, which produce catecholamine hormones. These hormones, which include dopamine, epinephrine (adrenaline) and norepinephrine, are commonly referred to as the ‘fight or flight’ hormones.

Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, bloodhello pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreas, and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cells, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response.

Paragangliomas are most commonly diagnosed in adults between the ages of 30 and 50. Hereditary paragangliomas are diagnosed equally in men and women; however, paragangliomas that develop without a family history have a higher incidence in women. It is important to note while it is more common in these demographics, anyone can develop this disease.

Types of Paragangliomas

There are two types of paragangliomas, which are categorised by the nerve cells they are associated with.

Sympathetic Paraganglioma

Sympathetic paragangliomas are functional tumours (tumours that produce and secrete hormones) that develop along the sympathetic chain in the body. The sympathetic chain consists of the bilateralBoth sides. nerves that run parallel to the spine from the base of the skull to the coccyx (bottom portion of the spine). The nerves in this chain are primarily responsible for the ‘fight or flight’ responses, such as sweat, reflexes and accelerated heart rate. These types of tumours generally have a higher rate of metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems. that spreads throughout the body) and tend to be highly symptomatic.

Parasympathetic Paraganglioma

Parasympathetic paragangliomas are non-functional tumours (tumours that do not produce and secrete hormones) that develop in the parasympathetic nerves of the body. The parasympathetic nerves are located in the brain and the sacral portion of the spine. The parasympathetic nerves act in contrast to the sympathetic nerves, and induce relaxation responses by slowing breathing and/or heart rate, lowering blood pressure and assisting in digestion and urination. These types of tumours are usually asymptomatic.

Treatment

If a paraganglioma is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

T (tumour) indicates the size and depth of the tumour.
N (nodes) indicates whether the cancer has spread to nearby lymphA clear fluid that moves through the body through the lymphatic system, carrying cells that fight infection. nodes.
M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0-IV:

Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedOnly to one area/place of body. cancer.
Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedAt a late stage, far along or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

Grade
• A score that tells how quickly a tumour might spread and grow by looking at how the abnormal cells and tissue look under a microscope.
• Grade is not the same as stage.
• Grading is different depending on the type of cancer.
I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.

Treatment is dependent on several factors, including location, stage of disease and overall health.

Treatment options for paragangliomas may include:

Surgery to remove as much of the tumour as possible (will vary based on location).
Radiation therapy, potentially including peptide receptor radionuclide therapy (PRRT).
Chemotherapy
• a chemical drug treatment to kill or slow-growing cancer Cells
• these drugs are called cytotoxic drugs
.
HormoneA chemical made in different body parts/organs that is sent out to other parts of the body through the bloodstream. Hormones watch over and help control how other cells or organs act. medications.
Targeted therapies.
Cryotherapy.
Watch and wait.
Clinical trials.
Palliative careLessening pain without curing the disease..

For more information on treatment options, please refer to the Rare Cancers Australia Treatment Options page.

Risk factors

The risk factors for paragangliomas are often associated with having certain medical conditions, including:

Multiple endocrine neoplasia 2 syndrome (MEN2A and MEN2B).
Von Hippel-Lindau (VHL) syndrome.
Neurofibromatosis type 1 (NF1).
Hereditary paraganglioma syndrome.
Carney-Stratakis dyad.
Carney triad.

Other risk factors for this disease include:

Family history of paragangliomas and/or pheochromocytomas.
ChronicA long-lasting disease that changes slowly. hypoxia (most commonly found in people living at high altitudes).
Mutations of the succinate dehydrogenase (SDH) gene.

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Some of the information regarding risk factors was obtained from the Paraganglioma page published by the National Cancer Institute.

Symptoms

Parasympathetic paragangliomas are often asymptomatic and can be misdiagnosed or go undetected.

Sympathetic paragangliomas may present with some of the following symptoms:

High blood pressure (hypertensionHigh blood pressure.).
Headaches.
Unexplainable sweatiness.
Accelerated heart rate.
Shakiness.
Being very pale.
Anxiety.
Heart palpitations.

Not everyone with the symptoms above will have cancer but, see your GP if you are concerned.

Diagnosis/diagnosing

If your doctor suspects you have a paraganglioma, they will order a variety of diagnostic tests to confirm the diagnosisUsing medical test results, identify and name a disease and/or condition., and refer you to a specialist for treatment.

Physical examination

Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.

Endocrine studies & blood tests

Endocrine studies involve blood and/or urine tests and imaging tests (see below) to analyse your hormone levels and detect any abnormalities. Some of these tests may include:

General blood test to assessTo measure, look at and learn from. overall health.
Blood catecholamine studies, which measure levels of certain catecholamine levels in your blood.
24-hour urine test, which measures levels of catecholamine levels in your urine.

Imaging tests

The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), ultrasounds, and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography scan.), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis. Your doctor may also recommend a somatostatin receptor scintigraphy scan, which involves injecting a radioactive substance into the bloodstream which attaches to the tumour and shows where the tumour(s) are located in the body.