Paraganglioma is a rare, benign tumor that develops from the specialized cells of the nervous system. A Paraganglioma tumor is usually slow-growing and non-cancerous (benign), but in some cases, it can be cancerous (malignant).
Most paraganglioma cancers occur in the head and neck region, but they can also occur in other parts of the body, such as the chest, abdomen, or pelvis. A Paraganglioma tumor is sometimes called a glomus tumor or chemodectoma.
Paraganglioma Causes and Risk Factors
The exact cause of Paraganglioma tumors is unknown, but there are several theories about what may contribute to their development. One hypothesis suggests that Paraganglioma tumors develop from the abnormal growth of neural crest cells. Neural crest cells are a type of cell that frequently develops into nerve tissue. Another theory recommends that Genetic mutations may cause paraganglioma tumors. Mutations in specific genes, such as the SDHB gene, have been linked to an increased risk of Paraganglioma tumors.
Paraganglioma tumors larger than 4 centimeters (cm) in diameter or spread to other parts of the body are more likely to be cancerous. Paragangliomas in the adrenal gland are also more likely to be cancerous.
There are numerous Paraganglioma tumors, and each type has its own set of risk factors. Head and neck paragangliomas (HNPs) are the most common type of paraganglioma. HNPs are more likely to occur in older adults, women, smokers, and people with a family history of the condition. Other risk factors for HNPs include exposure to certain chemicals, such as benzene or formaldehyde.
The second most common type of paraganglioma is extra-adrenal paragangliomas (EAPs). EAPs are more likely to occur in younger adults, men, and people with a family history of the condition. Other risk factors for EAPs include exposure to certain chemicals, such as arsenic or vinyl chloride.
Paraganglioma cancer can also occur in the adrenal gland (adrenal paragangliomas). Adrenal paragangliomas are more likely to occur in people with a family history of the condition.
Paraganglioma Symptoms and Diagnosis
The most common symptom of Paraganglioma tumors is a lump or mass in the head, neck, chest, or abdomen. Paraganglioma tumors can also cause other symptoms, such as:
- High blood pressure (hypertension)
- Rapid heartbeat (tachycardia)
- Abnormal heart rhythm (arrhythmia)
- Shortness of breath
- Wheezing
- Swelling of the extremities
- Pain in the head, neck, chest, or abdomen
- Weakness
- Numbness
- Paralysis
Paraganglioma tumors are usually diagnosed with a combination of imaging tests and biopsies.
- Imaging Tests for Paraganglioma Tumors: Imaging tests create pictures of the inside of the body. Imaging tests help diagnose Paraganglioma tumors because they can show the size, shape, and location of cancer.
- Biopsies for Paraganglioma Tumors: A biopsy is a procedure in which a small tissue sample is removed from the body and examined for cancer cells. Biopsies help diagnose Paraganglioma tumors because they can confirm the presence of cancer cells.
Paraganglioma Treatment
Paraganglioma treatment depends on the type, location, and size of the tumor, as well as your overall health and preferences. Paraganglioma tumors that are small and have not spread to other body parts can often receive treatment with surgery. Paraganglioma tumors that are larger or have spread to other parts of the body may require additional treatment, such as radiation therapy or chemotherapy.
Surgery for Paraganglioma Tumors: Surgery is the most common treatment for Paraganglioma tumors. The type of surgery you have will depend on the size and location of the cancer. Common types of surgery used to treat Paraganglioma tumors include:
- Local excision
- Partial removal
- Total removal
- Radical removal
Radiation Therapy for Paraganglioma Tumors: Radiation therapy is a treatment that uses high-energy beams to kill cancer cells. Radiation therapy can treat Paraganglioma tumors that are larger or have spread to other parts of the body.
Chemotherapy for Paraganglioma Tumors: Chemotherapy is a treatment that uses drugs to kill cancer cells. Chemotherapy can treat Paraganglioma tumors that have spread to other body parts.
Targeted Therapy for Paraganglioma Tumors: Targeted therapy targets specific molecules involved in the growth and spread of cancer cells. It can treat Paraganglioma tumors that have spread to other body parts.
Watchful Waiting for Paraganglioma Tumors: Doctors use a watchful waiting approach to monitor Paraganglioma tumors that have not spread to other body parts. No active treatment is given, but the cancer is closely monitored for changes.
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