An article in CURE® describes two extremely rare types of tumors: paraganglioma and pheochromocytoma. One in three thousand individuals may have either or both of these tumors.
The symptoms accompanying these tumors are quite common. So common that although U.S. President Dwight Eisenhower suffered from high blood pressure and headaches for most of his life, a pheochromocytoma tumor was not discovered until after his death.
About the Pheochromocytoma Tumor
The pheochromocytoma tumor is a neuroendocrine tumor that evolves from chromaffin cells within the adrenal gland.
Neuroendocrine tumors originate from cells that respond to signals they receive from the nervous system. They subsequently release hormones into the bloodstream. There is also the possibility of releasing excess hormones. An overabundance of hormones could be fatal.
About the Paraganglioma Tumor
Paraganglioma has a close relationship to pheochromocytoma although they are broken down further into nonchromaffin and chromaffin paragangliomas.
With respect to paraganglioma, nerve cells may be found at any point along the parasympathetic and sympathetic nervous systems.
Either paraganglioma or pheochromocytomas may be found to be metastatic, but this doesn’t happen in all cases.
Symptoms of Both Tumor types
- rapid heart rate
- flushing
- abdominal pain
- severe headaches
- heavy sweating
- high blood pressure
- anxiety
- joint pains
- pale skin
- weight loss
- emotional mood swings
Methods of Diagnosis
If a simple twenty-four-hour urine test detects an abnormal volume of any substance it generally signals disease. CT body scans of areas showing cell division, PET, and MRI scans are frequently used.
Additional Testing
- MIBG scan: used to locate neuroendocrine tumors
- metanephrines blood test
- somatostatin receptor scintigraphy: locates tumors
A Word of Caution
In an interview with Cure, Dr. Daniel Pryma, a division chief at Penn Medicine’s Molecular Imaging, explained that these tumors hold a tremendous blood supply. Therefore, to prevent excessive bleeding, Dr. Pryma recommends avoiding biopsies and bringing the patient directly to surgery.
Some Cases May be Hereditary
About thirty to forty percent of paraganglioma and pheochromocytomas may be inherited. If people carry the genetic mutation it presents a fifty percent risk of passing it to their offspring.
Treatment
Although surgery is currently standard procedure, there are several treatments available for patients who cannot undergo an operation or whose tumors have metastasized.
Only one medication, Azedra (iobenguane I 131), has been approved thus far by the FDA, but more studies are being conducted every year.
Dr. Pryma had another word of caution, this time about finding the right specialist. He explained that many doctors who do not have experience in treating these tumors may attend to them as they would attend to most cancers. This, he explains, may do more harm to the patient.
