|
Adrenal cancer is a rare disease that
originates in the adrenal glands. The adrenal
glands are located on top of the kidneys and
consist of two parts that function separately: the
outer layer (cortex) and the inner area (medulla).
The cortex produces three major hormones: cortisol
(a glucocorticoid), aldosterone (a
mineralocorticoid), and dehydroepiandrosterone
(DHEA; an androgen). The medulla produces
epinephrine (adrenaline), norepinephrine, and
dopamine.
Adrenal tumors can increase hormone production
(called functioning tumors). Adrenal tumors that
do not produce hormones are called nonfunctioning.
Symptoms of adrenal cancer and treatment for the
condition depend on whether the tumor is
functioning or nonfunctioning, and on which
hormone is being overproduced.
Types
Most (99%) adrenal tumors are noncancerous (i.e.,
benign) adrenal cortical adenomas and do not
require treatment. These tumors usually do not
cause symptoms, are small, and are found
incidentally during diagnostic imaging.
The most common type of adrenal cancer develops in
the adrenal cortex and is called adrenocortical
carcinoma. Functioning adrenocortical carcinomas
may produce symptoms related to increased hormone
production.
Nonfunctioning tumors may cause pain from pressure
on abdominal organs and a mass in the abdomen that
is able to be felt with the fingers (palpable).
Cancers that develop in the adrenal medulla
include neuroblastoma (originates in undeveloped
nerve cells) and pheochromocytoma (originates in
cells that produce epinephrine and
norephinephrine). Neuroblastoma usually occurs in
infants and children and pheochromocytoma more
commonly occurs in people who are in their 30s and
40s.
Other types of cancer (e.g., breast, lung) may
spread (metastasize) to the adrenal glands.
Incidence and Prevalence
Worldwide, about 1 out of 1 million people develop
adrenal cancer each year. Prevalence of the
condition is slightly higher in men in their 40s
and 50s and in children younger than 5 years old.
Causes and Risk Factors
The cause of adrenal cancer is unknown and most
cases do not have identifiable risk factors. In
some cases, heredity plays a role in the
development of the disease. Li-Fraumeni syndrome
and type 1 multiple endocrine neoplasia (MEN1) are
genetic mutations in tumor suppressor genes that
increase the risk for several types of cancer,
including adrenal cancer. Genetic testing may be
recommended in families with a high incidence of
suspected tumor suppressor gene mutation.
Other familial syndromes associated with adrenal
cancer include:
Gardner syndrome
Carney triad
Cowden syndrome
Familial polyposis
Turcot syndrome |
.jpg)
