faq’s

pheochromocytoma & paraganglioma

What is pheochromocytoma (pheo)?

What is pheochromocytoma (pheo)?

Pheochromocytoma is a neuroendocrine tumor. The tumor originates from chromaffin cells in the medulla (inside portion) of the adrenal gland. Pheo can secrete excessive amounts of catecholamines (hormones that make up adrenaline). Pathologists that suspect a tumor of chromaffin cells stain the tissue with chromic salts. If the tumor is pheo positive, the tissue will turn a dark dusky color. Hence the name, Pheochrome- (staining dark and dusky with chromic salts) -cytoma (a general term for a cellular tumor).

What is paraganglioma (para)?

What is paraganglioma (para)?

Paragangliomas are closely related to pheochromocytoma. However, paras originate from the ganglia (group of nerve cells) of the nervous system, allowing them to form anywhere along the sympathetic and parasympathetic nervous systems. There are two types of paragangliomas – chromaffin paragangliomas and nonchromaffin paragangliomas.

Chromaffin paragangliomas arise from chromaffin cells, and are sometimes called extra-adrenal pheochromocytoma, which means the tumor is found outside the adrenal gland. These tumors usually secrete hormones similar pheo. Chromaffin paras are rarer than pheo, but have a higher chance of malignancy (cancerous).

Nonchromaffin paragangliomas are usually named based on the primary anatomical site of origin. For example, a Carotid Body Tumor (CBT) is another name for a neck paraganglioma. Nonchromaffin paras arise from glomus cells instead of chromaffin cells. They are commonly noncancerous, and generally present in the head and neck areas. Nonchromaffin paras rarely secrete hormones, and can occur within familial lines.

Is it cancer?

Is it cancer?

Pheo and Para are often said to be benign tumors. Meaning that after the one tumor is surgically removed, it does not spread (metastasize) or come back (recur). However, some pheos and para have higher metastic (cancerous) potential. Many experts advise to have routine scans and tests for several years after a pheo or para has been removed.

Which of the following are symptoms of pheochromocytoma and paraganglioma?

Which of the following are symptoms of pheochromocytoma and paraganglioma?

Functioning pheo and para (meaning pheos and paras that secrete the excessive catecholamines) can produce multiple symptoms. Some patients experience all symptoms on the list, while others may only experience one or two. Those symptoms include (but are not limited to):

  • High blood pressure
  • Rapid heart rate / forceful heartbeat
  • Profound Sweating
  • Flushing
  • Abdominal pain
  • Sudden-onset of headaches (usually severe)
  • Anxiety
  • Emotional mood swings
  • Joint pains
  • Pale Skin
  • Weight loss

Due to the fact that many symptoms are common in many other ailments, pheo has been nicknamed, “The Great Mimic,” and while many pheo patients experience high blood pressure, pheo only counts for about 1% of high blood pressure cases. That means most people with high blood pressure will not have pheo.

Non-Functioning pheos and paras may not yield the same symptoms as functioning pheos and paras. They often go undetected, are found incidentally, or start causing symptoms like pain or swelling of surrounding organs when tumors grow too large. For instance, Carotid Body Tumors (neck paras) are often found by seeing or feeling a lump in the neck before other symptoms like pain or difficulty swallowing occurs.

What are the dangers to pheo and para?

What are the dangers to pheo and para?

Functioning pheo and para create excessive amounts of catecholamines in a body, elevating the risk of heart attack, stroke, and even kidney failure. For patients with functioning pheos and para, there is also a danger to outpatient procedures that involve anesthesia; this includes dental procedures that involve certain numbing agents. A reaction to the anesthetics causes the body to release a large amount of catecholamines that can send the patient into a catecholamine crisis and cause stroke or heart attack.

Even non-functioning tumors pose a threat by growing too large and/or causing nerve and organ damage, and because they are made of the same cells as functioning tumors, they can “switch on” at any time causing all the dangers that a functioning pheo and para patient experience.

How common is pheochromocytoma and paraganglioma?

How common is pheochromocytoma and paraganglioma?

According to the National Cancer Institute (U.S.) 2 to 8 people per million are diagnosed with pheo each year. Para is said to be even rarer. Many doctors will go their entire career without ever seeing a pheo or para patient.

Is it Genetic? (Can I pass this down to my children?)

Is it Genetic? (Can I pass this down to my children?)

Genetic studies of pheo and para are constantly evolving. Within the last five years, a large number of genetic links to pheo and para have been identified. If this continues, leading experts have predicted that in the next decade we could see up to 50% of the pheo and para cases be linked to a genetic cause. Whether that cause can be passed down in the families depends solely on the type of genetic mutation a patient has. To learn more about the genetics behind pheo and para, visit our genetics section of the site.

Is there a cure for paraganglioma or pheochromocytoma?

Is there a cure for paraganglioma or pheochromocytoma?

The only cure to para and pheo at this time is surgical removal of the tumor. There are a few treatment options available to patients that cannot be operated on, or have tumor metastasis (cancer), and there are more studies and research being done each year to find a cure. To find out more about the known treatment options, visit our Treatments and Trials section of the site.

If it is not pheo or para, what could it be?

If is is not pheo or para, what could it be?

Given its rarity and the fact that pheo is called, “The Great Mimic” there are many ailments and diseases that have similar symptoms of pheo and para. One of the best resources that list all the differential diagnoses can be found here:

Differential Diagnosis of Pheochromocytoma

Endocrine

  • adrenal medullary hyperplasia
  • hyperthyroidism, thyroid storm
  • carcinoid
  • hypoglycemia (often due to the presence of insulinoma)
  • medullary thyroid carcinoma
  • mastocytosis
  • menopausal syndrome

Cardiovascular

  • heart failure
  • arrhythmias
  • ischemic heart disease, angina pectoris
  • baroreflex failure
    • a. syncope
    • b. orthostatic hypertension
    • c. labile hypernoradrenergic essential hypertension
    • d. renovascular disease

Neurologic

  • migraine or cluster headaches
  • stroke
  • diencephalic autonomic epilepsy
  • meningioma
  • POTS (postural orthostatic tachycardia syndrome)
  • Guillain-Barre syndrome
  • encephalitis

Psychogenic

  • anxiety or panic attacks
    • a. factitious use of drugs
    • b. somatization disorder
    • c. hyperventilation

Pharamacologic

  • tricyclic antidepressant
    • a. cocaine
    • b. alcohol withdrawal
    • c. drugs stimulating adrenergic receptors
    • d. abrupt clonidine withdrawal
    • e. dopamine antagonists
    • f. monoamine oxidase inhibitors
    • g. ephedrine-containing drugs
    • h. factitious use of various drugs including catecholamines

Miscellaneous

  • neuroblastoma, ganglioneuroma, ganglioneuroblastoma
  • acute intermittent porphyria
  • mastocytosis
  • unexplained flushing spells
    • a. recurrent idiopathic anaphylaxis
    • b. lead and mercury poisoning