In some cases, pheochromocytoma can run in families. About 25-35% of cases of pheochromocytoma may be inherited. Some of these inherited cases may be associated with a genetic condition, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis.
If you have a pheochromocytoma, the tumor releases hormones that may cause high blood pressure, headache, sweating and symptoms of a panic attack. If a pheochromocytoma isn't treated, severe or life-threatening damage to other body systems can result.
Tumor that forms in the center of the adrenal gland (gland located above the kidney) that causes it to make too much adrenaline. Pheochromocytomas are usually benign (not cancer) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.
Although pheochromocytomas have classically been associated with 3 syndromes—von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN 2), and neurofibromatosis type 1 (NF1)—there are now 10 genes that have been identified as sites of mutations leading to these tumors.
The clinical hallmark of pheochromocytoma is hypertension, but some patients are normotensive and may even be hypotensive.
If left untreated, pheochromocytomas/paragangliomas may progress to cause serious, life-threatening complications including heart muscle disease (cardiomyopathy), bleeding in the brain (cerebral hemorrhaging) or the accumulation of fluid in the lungs (pulmonary edema).
Patients with a small pheochromocytoma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with pheochromocytoma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.
The most common site of occurrence of extra-adrenal pheochromocytoma is the superior para-aortic region between the diaphragm and lower renal poles.
The Slippery Symptoms of Pheochromocytoma
Symptoms of pheochromocytoma can include high blood pressure, a rapid heartbeat, chest pain or pressure, profuse sweating or flushing, severe headaches, anxiety, and abdominal pain.
President Eisenhower experienced an acute heart attack in September 1955 and died of ischemic cardiomyopathy 14 years later. The autopsy revealed, unexpectedly, a 1.5-cm pheochromocytoma in the left adrenal gland.
Recovery from a pheochromocytoma or paraganglioma is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.
According to the “rule of 10”, in 1/10 patients with pheochromocytoma it is malignant, in 1/10 of cases the tumor is bilateral, in 1/10 extra-adrenal and in 1/10 familial. Surgical resection, the only curative treatment, carries a high risk of hypertensive crises due to massive catecholamine release.
Even benign adrenal tumors can be dangerous or cause uncomfortable symptoms. Adrenal tumors can be malignant (cancer) or benign (not cancerous). Even benign adrenal tumors can be dangerous or cause uncomfortable symptoms.
Anxiety and panic disorder may be the main presenting symptoms of pheochromocytoma.
Pheochromocytoma is suspected in patients with typical symptoms or particularly sudden, severe, or intermittent unexplained hypertension. Diagnosis involves demonstrating high levels of catecholamine products in the serum or urine.
Because catecholamine relase varies throughout the day, the best method of diagnosing pheochromocytomas is using a 24-hour urine collection. This involves obtaining a special urine container, which has a small amount of preservative, from a medical laboratory and filling it with one entire day's worth of urine.
Symptoms from a pheochromocytoma are often characterized by paroxysm (“spells”). This means patients develop symptoms from the pheo during an attack, a “spell”, which often last 5 to 20 minutes. The typical spell is associated with very high blood, headache, palpitations (heart racing), and sweating.
Pheochromocytoma is a rare tumor, which can present with unusual manifestations such as severe abdominal pain with elevated liver enzymes.
Pheochromocytomas affect men and women equally. The tumor can be diagnosed at any age, but it is most common between the ages of 30 and 50. Approximately 10% of these tumors are diagnosed in children. Age at diagnosis depends on whether the tumor is related to an inherited syndrome.
Death resulting from unrecognised or untreated tumour is caused by cardiovascular complications.
Pheochromocytomas are rare, occurring in about 2 to 8 out of every one million people. Approximately 10% of patients are found to have pheochromocytomas in both adrenal glands, which is most commonly seen in younger patients with pheochromocytoma-related genetic syndromes.
Agents known to provoke a pheochromocytoma paroxysm (eg, beta-adrenergic blocker in absence of alpha-adrenergic blockade, glucagon, histamine, metoclopramide, high-dose corticosteroids) should be avoided.
A pheochromocytoma's long-term consequences can include cardiac muscle deterioration, congestive heart failure (CHF), a higher diabetes risk and possibly death. Masked hypertension (MH) is more common in people with adrenal pheochromocytoma, which has been related to an increased risk of heart disease.