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HomeHealth articlesretroperitoneal paragangliomaWhat Is Retroperitoneal Paraganglioma in the Extra-Adrenal Gland?

Retroperitoneal Paraganglioma in the Extra-Adrenal Gland - Causes and Treatment

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Retroperitoneal paraganglioma in the extra-adrenal gland is a rare tumor, and the article below shares more information about the same.

Medically reviewed by

Dr. Shivpal Saini

Published At February 24, 2023
Reviewed AtOctober 5, 2023

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:

  • Multiple Endocrine Neoplasia 2 Syndrome: It is a genetic disorder that mainly affects the body's endocrine glands.

  • 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.

  • Type 1 Neurofibromatosis (NF1): Genetic disorder affecting the skin and causing discoloration.

  • Hereditary Paraganglioma Syndrome: Genetic disorder increases the risks of developing paragangliomas.

  • Carney-Stratakis Dyad: Genetic disorder in which paraganglioma forms along with gastrointestinal stromal tumor (GIST).

  • 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:

  • High blood pressure is one of the common paraganglioma symptoms (hypertension).

  • A person will experience frequent headaches.

  • Excessive sweating without apparent cause.

  • A rapid, hammering, or unsteady heartbeat.

  • Feeling unsteady.

  • The person will appear paler than usual.

  • Vomiting and nauseous.

  • Diarrhea.

  • Constipation.

  • Elevated levels of blood sugar (hyperglycemia).

  • A sharp fall in blood pressure upon abruptly standing (orthostatic hypotension).

  • Unexplained weight loss.

  • Some paraganglioma patients may only occasionally or sporadically experience symptoms.

How Are Retroperitoneal Paraganglioma Diagnosed?

  • 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.

  • 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.

  • 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:

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • Heredity syndrome of paraganglioma or pheochromocytoma.

  • Syndrome of multiple endocrine neoplasia type 2.

  • Von Hippel-Lindau disease (VHL).

  • Type 1 neurofibromatosis.

  • Carney-Stratakis dyad.

  • 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.

Frequently Asked Questions

1.

What Does Retroperitoneal Paraganglioma Mean?

Paraganglioma refers to tumors that develop from extra-adrenal chromaffin cells. They arise from paraganglia at various anatomical sites, including the abdomen, head, neck, and thorax. Retroperitoneal paragangliomas are tumors located close to the renal (kidney) arteries and veins surrounding the inferior vena cava and the abdominal aorta.

2.

Can Paraganglioma Cause Cancer?

Paragangliomas are usually benign (noncancerous) and are slow growing. But, some paragangliomas have the risk of becoming malignant (cancerous). In addition, benign paragangliomas have the risk of causing cancer. Approximately 20 percent of paragangliomas are cancerous.

3.

How Long Can a Person With Paraganglioma Live?

Patients with paraganglioma, which is smaller in size and has not spread to other body parts, have a five-year survival rate of about 95 percent. Patients with paraganglioma that recurred or spread to other body parts have a five-year survival rate between 34 percent and 60 percent.

4.

Why Does Paraganglioma Occur?

The exact cause for paraganglioma has not been established. Some paragangliomas are caused by genetic mutations transmitted from parents to their children. Approximately 25 to 35 percent of individuals with paraganglioma have hereditary conditions linked to paraganglioma.

5.

What Is the Prognosis for Paraganglioma?

The prognosis of paraganglioma depends on the size of the tumor, the location of the tumor, the nature of the tumor, its ability to spread, and the amount of tumor resected during surgery. Individuals with a small paraganglioma that has not spread to other body parts have a five-year survival rate of 95 percent. Individuals with tumors that have recurred or spread to other body parts have a five-year survival rate between 34 percent and 60 percent.

6.

What Is the Common Site of a Paraganglioma?

Paragangliomas are commonly seen in the pre-aortic and paravertebral sympathetic plexus or the skull base. Paragangliomas are commonly seen in the head and neck region near the carotid artery and in other body parts. Paragangliomas in the jugular foramen, carotid body, or ear are less differentiated tumors.

7.

Can Paraganglioma Cause Serious Complications?

Paragangliomas are usually noncancerous, but some can cause cancer and spread to other body parts. The survival rate depends on the stage and location of paraganglioma, a person’s age, and overall health. If left untreated, paraganglioma can cause life-threatening complications.

8.

Is Paraganglioma Considered a Disease or a Disorder?

Paragangliomas are tumors that develop near the carotid artery in the head and neck region and other body parts. Some paragangliomas secrete catecholamines called adrenaline and noradrenaline. Releasing these catecholamines into the blood can cause signs or symptoms of a disease. Hence, paragangliomas are considered a disease.

9.

How Is Paraganglioma Diagnosed?

Paragangliomas are diagnosed through radiographic imaging techniques. Anatomical and functional imaging is required for the diagnosis and staging of paragangliomas. Most tumors are readily detected using In-111 scintigraphy, which helps detect additional lesions and other metastatic foci.

10.

What Is the Other Name for Paraganglioma?

Paraganglioma is also referred to as extra-adrenal pheochromocytoma. Paragangliomas develops in the nerve tissue in the adrenal gland and near specific nerves and blood vessels. Paragangliomas that develop in the adrenal glands are referred to as pheochromocytomas.

11.

What Is the Growth Rate of a Paraganglioma?

Paragangliomas are usually slow-growing tumors. But, the growth pattern varies by person. In addition, the growth pattern of paragangliomas is not related to tumor location and size. Though these tumors are slow-growing, they can cause cancer and spread to other body parts.

12.

Do Paraganglioms Recur?

Paragangliomas can recur even after proper treatment. The tumor may recur in the same place or other body parts. The tumor can recur even after several years of initial treatment. Recurrence depends on the tumor location, size, and the amount of tumor removed during surgical resection.
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Dr. Shivpal Saini
Dr. Shivpal Saini

General Surgery

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