Introduction
Retroperitoneal paraganglioma of the extra-adrenal gland is a type of paraganglioma usually seen in the abdomen. Extra-adrenal paragangliomas are tumors that grow outside of the adrenal glands and make and release adrenaline, a hormone that helps control blood pressure. On the other hand, pheochromocytomas are tumors that also produce adrenaline but develop in the center of the adrenal glands.
The adrenal glands are found on top of each kidney. They are part of the endocrine system. The adrenal glands produce catecholamine hormones like epinephrine (adrenaline) and norepinephrine (noradrenaline). Adrenaline and noradrenaline form the body's sympathetic nervous system. The main job of the sympathetic nervous system is to regulate the body's organs. Extra-adrenal paragangliomas typically develop in the stomach, although they can also develop in the head, neck, and thoracic areas. They are more malignant (harmful) than pheochromocytomas.
What Is the Cause of Retroperitoneal Paraganglioma Development?
The cause of paraganglioma is typically unknown, and it develops randomly. However, a genetic disorder associated with paragangliomas includes:
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Multiple Endocrine Neoplasia 2 Syndrome: It is a genetic disorder that mainly affects the body's endocrine glands.
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Von Hippel-Lindau Disease (VHL): It is a genetic disorder that produces tumors at several locations in the body, such as the brain, spinal cord, eyes, kidney, and adrenal gland.
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Type 1 Neurofibromatosis (NF1): Genetic disorder affecting the skin and causing discoloration.
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Hereditary Paraganglioma Syndrome: Genetic disorder increases the risks of developing paragangliomas.
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Carney-Stratakis Dyad: Genetic disorder in which paraganglioma forms along with gastrointestinal stromal tumor (GIST).
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Carney's Triad: This triad includes paraganglioma, GIST, and pulmonary chondroma.
What Are the Symptoms of Retroperitoneal Paraganglioma?
When the tumor produces too much dopamine, adrenaline (epinephrine), or noradrenaline (norepinephrine) in the blood, signs and symptoms of paraganglioma appear. But not all paraganglioma tumors make too much dopamine, noradrenaline, or adrenaline and cause symptoms. These tumors are asymptomatic. Some people may exhibit the following symptoms:
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High blood pressure is one of the common paraganglioma symptoms (hypertension).
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A person will experience frequent headaches.
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Excessive sweating without apparent cause.
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A rapid, hammering, or unsteady heartbeat.
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Feeling unsteady.
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The person will appear paler than usual.
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Vomiting and nauseous.
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Diarrhea.
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Constipation.
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Elevated levels of blood sugar (hyperglycemia).
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A sharp fall in blood pressure upon abruptly standing (orthostatic hypotension).
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Unexplained weight loss.
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Some paraganglioma patients may only occasionally or sporadically experience symptoms.
How Are Retroperitoneal Paraganglioma Diagnosed?
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Blood and Urine Tests: These tests are used to diagnose paragangliomas. To determine the hormone levels in the body, the doctor may advise blood and urine tests. These examinations may find an elevated hormone level secreted by paraganglioma cells or chromogranin (a tumor marker) in blood or urine.
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Imaging Scan: Imaging tests are beneficial to visualize the size of paraganglioma better and help doctors choose the most effective treatments. Imaging methods that could include MRI (magnetic resonance imaging), CT (computed tomography) scan, and specialized nuclear medicine imaging, including a positron emission tomography (PET) scan and a metaiodobenzylguanidine (MIBG) scan, provide better visualization of the paraganglioma.
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Genetic Analysis: Gene mutations transferred from parents to children occasionally can cause paragangliomas. Genetic testing is recommended to identify these mutations.
How Is Retroperitoneal Paraganglioma Treated?
If a paraganglioma makes too many hormones, some medicines and lifestyle changes can help lower hormone levels and control symptoms. Before starting paraganglioma treatment, the hormone levels must be brought under control. The drugs alpha-blockers, beta-blockers, and calcium channel blockers regulate hormone levels. Other approaches include eating sodium-rich foods and drinking lots of fluids. Treatment methods may include:
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Surgery: Paraganglioma can be removed by surgery, and the doctor may suggest it be done. Before that, the doctor may prescribe medications to control the excessive hormones produced by paraganglioma. Excess hormones may be released during surgery.
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Radiation Therapy: High-energy beams like X-rays and protons stop tumors from growing. It can be used to treat pain due to paragangliomas that have spread to other areas of the body. Stereotactic body radiotherapy is a type of radiation therapy that uses many radiation beams that are all pointed at the tumor. The beams target the paraganglioma cells and minimize the damage to nearby healthy tissues.
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Thermal Ablation Therapy: Heat or cold is used in thermal ablation therapy to kill tumor cells and stop tumor growth. Radiofrequency ablation is a type of thermal ablation therapy in which electrical energy is used to heat the tumor cells. Cold gas is used in a different treatment called cryoablation to freeze the tumor cells.
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Chemotherapy: Chemotherapy uses medications to kill proliferating cells, such as cancer cells, rapidly. It helps shrink tumor growth. Before starting chemotherapy, the patient is given medications to regulate hormone levels of paraganglioma if hormones are found to be high.
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Targeted Drug Therapy: Targeted medication therapies focus on specific defects in cancer cells. Targeted drug therapies either stop cancer cells from multiplying or slow them down by getting in the way of how tumors grow. Medications targeting paraganglioma cells with radioactive material can send radiation straight to the tumor cells. These therapies might be an option for paragangliomas that metastasize to different body regions.
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Wait and Watch: In other cases, such as when a paraganglioma is slow-growing and not exhibiting any symptoms, doctors do not give any treatment. Instead, doctors tell patients to monitor their health and come for regular follow-up sessions.
How Is Retroperitoneal Paraganglioma Prevented?
Unfortunately, the development of paraganglioma cannot be stopped. However, genetic counseling can assist in screening for paraganglioma. However, an individual at high risk of developing paraganglioma due to inherited (it runs in the family) disorders and genes, genetic counseling may help detect the disease in its early stages. Therefore, an individual with first-degree relatives (parents, siblings) with any of the following inherited diseases should talk to a doctor.
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Heredity syndrome of paraganglioma or pheochromocytoma.
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Syndrome of multiple endocrine neoplasia type 2.
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Von Hippel-Lindau disease (VHL).
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Type 1 neurofibromatosis.
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Carney-Stratakis dyad.
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Carney triad.
Conclusion
Paraganglioma is an abnormal cell growth that starts in a certain nerve cell all over the body. Chromaffin cells, a particular type of nerve cell, play a critical role in the body, including controlling blood pressure. Tumors of the adrenal glands are called pheochromocytomas. The tumors are known as paragangliomas when they develop elsewhere in the body other than the adrenal gland. A retroperitoneal paraganglioma is a rare form of cancer; although it can happen at any age, adults between the ages of 30 and 50 are prone the most.
Most paragangliomas have no known cause, but they are passed down from generation to generation because of changes in genes. One of the catecholamines that paraganglioma cells often release is adrenaline. This hormone controls the "fight or flight" response. It can cause episodes of high blood pressure, a fast heartbeat, sweating, headaches, and tremors. Surgery to remove the tumor is the most common treatment for paragangliomas. However, if the paraganglioma is cancerous or has spread to other parts of the body, it might be time to think about more treatments. Normally paragangliomas are noncancerous; however, if they are cancerous, the current treatment regimen helps reduce the tumor size and increase the survival rate.
