Pheochromocytoma is a rare tumor that usually starts in the cells of one of your adrenal glands (glands on top of the kidneys).
Their frequency is
about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered
adrenal mass.
The cause is unknown although valuable
information has recently been provided by work on the genomics of familial diseases
including these tumors.
Although they are usually benign,
pheochromocytomas often cause the adrenal gland to make too many
hormones (dopamine, epinephrine and norepinephrine). This can lead to high blood pressure, diabetes and cause symptoms such
as
-
Headaches
-
Sweating
-
Pounding of the heart (palpitations)
-
Being shaky
-
Being extremely pale
- Weight loss
Sometimes pheochromocytoma is part of another
condition called multiple endocrine neoplasia syndrome (MEN) and von Hippel-Lindau
disease. People
with MEN often have other cancers and other problems involving hormones.
Doctors use lab tests and imaging tests to diagnose it.
The purpose of pre-operative imaging tests is to locate the tumor, ascertain whether
it is single or multiple, adrenal or ectopic (outside the adrenal gland), benign or malignant, and isolated or
present with other neoplasms (tumors) in the context of familial syndromes.
Surgery
is the most common treatment. Other options include radiation therapy,
chemotherapy, and targeted therapy. Targeted therapy uses substances
that attack cancer cells without harming normal cells.
Source:
Orphanet Journal of Rare Diseases
