Pheochromocytoma (cont.)
What are the symptoms of a pheochromocytoma?
Someone with a pheochromocytoma usually has 3 classic symptoms -- headache,
sweating, and heart palpitations (a fast heart beat).
When people go to their doctors with these symptoms,
high blood pressure may be found. In some cases, the high blood pressure comes
and goes and may be difficult to document. In other cases, the blood pressure is consistently
elevated and easily recorded.
Pheochromocytomas, as I have mentioned, are not common.
They are present in only about 0.2% of all people with high blood pressure.
There are certain instances, however, where the diagnosis of pheochromocytoma may rank high on the
list of possibilities.
What conditions are associated with pheochromocytomas?
Certain families have an increased risk of
pheochromocytomas. The most common familial condition is called multiple endocrine neoplasia, or MEN for short. Two
types of MEN -- MEN 2A and 2B -- are associated with pheochromocytomas. Both are
genetic syndromes that run in families and are transmitted from parent to child
in an autosomal dominant manner.
Pheochromocytomas are not the only tumors that occur in MEN 2A and 2B. MEN 2A
carries an increased risk of tumors of the parathyroids, glands near the thyroid
that help to regulate calcium. And both MEN
2A and 2B elevate the risk of thyroid cancer. In families where MEN is suspected, genetic testing can be
done today to help identify family members at risk.
There are a number of other genetic disorders associated with
pheochromocytomas. The most prominent is called von Hippel-Lindau syndrome. This disorder is rare but important because it is associated with
the development of
numerous benign and malignant tumors.
There are also many individuals who have
pheochromocytomas with no known family history of them. These cases are termed
sporadic. In general, if these patients have bilateral disease
(pheochromocytomas in both adrenal glands) or
are diagnosed before the age of 21, genetic screening is recommended.
Next: What else can cause the symptoms of a pheochromocytoma? »
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