tumors are extremely rare with only 150-300 new cases per year. The adrenal
glands are located on top of the kidneys, consisting of two parts…the inner
area, called the medulla, and the outer area, called the cortex. The job of the
adrenal glands is to produce hormones:
cortex produces three major hormones:
(DHEA; an androgen).
tumors may or may not secrete hormones, which can cause significant symptoms.
The Cushings syndrome results in a swollen face, obesity, high-blood pressure,
sugar metabolism problems, and nerve problems. Other tumors may cause hair
growth (male patterns) in women or breast swelling in men. The most common type
of adrenal cancer is adrenocortical carcinoma. Some cancers, such as breast
cancer or lung cancer may metastize to the adrenal glands, but are still treated
as the "primary cancer"…breast or lung. 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
is covered in its own chapter herein. Pheochromocytoma more commonly occurs in
people who are in the 30 to 40 year old group.
require treatment before surgery (neoadjuvant treatment) for high blood
pressure, which often includes alpha-blockers (e.g., phenoxybenzamine, prazosin)
followed by beta-blockers (e.g., propranolol), and metyrosine.
for pheochromocytomas are as follows:
urinary catacholamines and metanephrines: This study is designed to measure
production of the different types of adrenaline compounds that the adrenal
makes. Since the body gets rid of these hormones in the urine, the patient's
urine is collected for 24 hours to determine if there is an over-production in
adrenaline compounds. This test measures different types of adrenaline
(epinephrine, norepinephrine, dopamine) as well as the break-down products of
these compounds which the liver and kidney have degraded. Since these compounds
are concentrated in the urine, this test is very good at making the diagnosis of
catacholamines (Blood Test): This study measures adrenaline compounds in the
blood. It is not as sensitive a test for pheochromocytoma as the 24-hour urine
test (sometimes the urine test will be positive and the blood test will be
negative), but it still can give important information if it shows elevated
Tests for Pheochromocytomas: There are 4 primary x-ray tests to examine the
adrenal glands (and the rest of the abdomen) for the presence of a
Treatment of Pheochromocytomas:
pheochromocytomas should be removed surgically. The vast majority of patients
can be treated with the new technique of minimally invasive Laparoscopic
Adrenalectomy. This is now the preferred method for removing
pheochromocytomas and is available in most hospitals in the U.S.
the surgical removal of the adrenal gland, is the only cure for adrenal cancer.
It is important to determine if the cancer has spread before surgery, because
metastases to lymph nodes or other organs (liver, lungs, kidneys) often requires
extensive surgery. Large adrenal tumors that have not spread are sometimes
removed using general surgical procedures.
is a treatment that travels throughout the body via the bloodstream (called a
systemic treatment) that often uses a combination of drugs to destroy cancer
cells. It is used as a palliative treatment for metastatic adrenal cancer and
may also be used in addition to surgery (adjuvant therapy). Drugs may be
administered orally or through a vein (intravenously).
(Lysodren®) suppresses adrenal gland function and is the drug of choice to
treat inoperable adrenal cancer. Approximately 20% of adrenal cancer patients
respond to treatment with mitotane, though side effects, which include
gastrointestinal disturbances (loss of appetite, nausea, vomiting, diarrhea) and
neurological disturbances (depression, lethargy, sleepiness), can be severe.
When mitotane therapy fails, cisplatin (Platinol®) may be tried, alone or
combined with other agents such as cyclophosphamide (Cytoxin®, Neosar®),
doxorubicin (Adriamycin®), cisplatin, fluorouracil (Adrucil®, Efudex®),
doxorubicin, cisplatin® Cisplatin with VP-16
Therapy uses high energy x-rays to destroy cancer cells. Radiation is not
used as a primary treatment for adrenal cancer. It is sometimes used as a pain
relieving (palliative) treatment for metastatic adrenal cancer.
Management for patients with functioning tumors includes managing symptoms
caused by increased hormone production. Increased cortisol production, know as
Cushing's syndrome, is often treated with aminoglutethimide or ketoconazole
(Nizoral®) to inhibit cortisol build-up (synthesis). They may be used alone, or
in combination with chemotherapy. Side effects include nausea, vomiting, and
aldosterone production, called Conn's syndrome, is usually treated using
spironolactone (Aldactone®). Spironolactone is an aldosterone antagonist (it
counteracts the action of aldosterone). Side effects include ulcers, abnormal
breast enlargement in men (gynecomastia), fever, and headache.
inhibitors such as anastrozole (Arimidex®) and anti-androgens such as
bicalutamide (Casodex®) may be used to treat excessive androgen production.
Pheocyhromocytomas are rare tumors of the adrenal glands that may secrete
catacholamine hormones including norepinephrine and/or adrenaline. These
tumors may produce high-blood pressure, headaches, nausea, sweating, anxiety,
Pheochromocytoma, a rare cancer, is a disease in which cancer (malignant)
cells are found in special cells in the body called chromaffin cells. Most
pheochromocytomas start inside the adrenal gland (the adrenal medulla) where
most chromaffin cells are located. There are two adrenal glands, one above
each kidney in the back of the upper abdomen. Cells in the adrenal glands make
important these important hormones to help the body function correctly.
Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may
also start in other parts of the body, such as the area around the heart or
bladder. Most tumors which start in the chromaffin cells are benign tumors,
and do not spread to other parts of the body and are not cancer. If a tumor is
found, the doctor will need to determine whether it is cancer or benign.
Pheochromocytomas often cause the adrenal glands to make too many hormones
called catecholamines. The extra catecholamines cause the high blood pressure
(hypertension), which can cause headaches, sweating, pounding of the heart,
pain in the chest, and a feeling of anxiety. High blood pressure that goes on
for a long time without treatment can lead to heart disease, stroke, and other
major health problems.
Diagnosis and prognosis can vary with each patient. Pheochromocytoma is
sometimes part of a condition called multiple endocrine neoplasia syndrome
(MEN). People with MEN often have other cancers (such as thyroid cancer) and
other hormonal problems. The chance of recovery depends on how far the cancer
has spread, and the patient's age and general health. If there are symptoms, a
doctor may order blood and urine tests to see if there are extra hormones in
the body. The doctor may use a CT scan, or an MRI scan, to make a picture of
the abdomen and other internal organs, which may be helpful in making a
Surgery is the most common treatment of pheochromocytoma. A doctor may
remove one or both adrenal glands in an operation called adrenalectomy. The
doctor will look inside the abdomen to make sure all the cancer is removed. If
the cancer has spread, lymph nodes or other tissues may also be taken out.
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by
pill, or it may be put into the body by a needle in the vein or muscle.
Chemotherapy is called a systemic treatment because the drug enters the
bloodstream, travels through the body, and can kill cancer cells throughout
Radiation therapy uses high-energy x-rays to kill cancer cells and shrink
tumors. Radiation comes from a machine outside the body (external radiation
The newest clinical trial for pheochromocytoma will evaluate the
effectiveness of Iodine 131MIBG (I-131MIBG) in treating malignant
pheochromocytoma and whether sensitization medications improve the response to
treatment. I-131 MIBG is metaiodobenzylguanidine, a taget agent fused with
iodine 131. Pheochromocytoma is a rare type of tumor that usually occurs in
the adrenal glands. The tumor cells release chemicals like adrenaline that can
cause large increases in blood pressure and pulse rate, with serious health
consequences. Tumor in the adrenal glands usually can be removed surgically,
but if the pheochromocytoma has spread to many sites in the body, or is
located in places where surgery is difficult or impossible, no satisfactory
treatment is available. I-131MIBG is a combination of an adrenaline-like
chemical and a radioactive form of iodine. The I-131MIBG attaches to the tumor
cells and the high concentration of radioactive iodine kills them. Previous
studies using 131MIBG to treat pheochromocytoma had a 36% response rate in
terms of complete or partial improvement. This study will examine whether
adding other sensitization medications to the 131MIBG treatment regimen will
enhance its effectiveness in reducing the size and number of tumors.