J.T. Nguyen1, B.L. Chia2, T.N.

Ha3
1,2, 3 University of South Australia, Adelaide, AUSTRALIA

To understand pheochromocytoma and its etiology, epidemiology, signs and symptoms, diagnosis,
staging, imaging, treatment and prognosis. Dependent on the presentation of significant catecholamine excess. Levels of catecholamine and its
metabolites can be measured through either blood or urine tests 4.

Urine test 7:
The adrenal glands are triangular shaped endocrine glands that sit on top of the kidneys. The adrenal • Gold standard for diagnosis because catecholamines are released throughout the day and the levels
glands are made up of 2 parts; adrenal cortex and medulla. Within the medulla, it contains vary
neuroendocrine cells known as chromaffin cells which are also found in other ganglia of the • Healthy people excrete only very small amounts
sympathetic nervous system. • May be inconvenient
RadioGraphics 2004 RadioGraphics 2004
•http://emedicine.medscape.com/article/379861-imaging
Pheochromocytoma is a rare cancer of the adrenal glands and it arises from chromaffin cells. As • Gives the highest reliability and specificity results
Fig. 3 Abdominal Aortogram Fig. 4 T1-weighted axial MRI Fig. 5 T2-weighted coronal MRI
pheochromocytomas usually arise from chromaffin cells, they are also called chromaffin tumours. 90%
of pheochromocytomas are typically located in the adrenal medulla of the adrenal glands. However, Blood (plasma) test 8:
this tumour can also be found outside the adrenal glands such as the heart and bladder and these • More convenient than the urine test Radiographs tend to have many limitations in imaging pheochromocytomas. However, large
chromaffin cancers are referred to as paragangliomas.
• Less sensitive method adrenal masses compressing the upper pole of the kidney may be discovered incidentally on
• Usually gives false positive results intravenous urograms and abdominal aortograms (Figure 3). In some cases, enlargement of the
Adrenal gland function to secrete the epinephrine and norepinephrine hormones, also known as
adrenal medullary may be seen on an abdomen x-ray due to a pheochromocytoma; however
adrenaline and nor-adrenaline. When adrenaline is released from the adrenal gland into the blood
these are all incidental findings.
stream, its function is to help regulate factors in the body such as blood pressure and heart rate. Imaging studies are usually done after the diagnosis to look at the adrenal glands if the levels of
Pheochromocytomas cause the adrenal medulla to secrete too much adrenaline resulting in high blood epinephrine, norepinephrine and their metabolites are greater than 2 times the normal level.
pressure. This disease can be fatal if left unrecognised as it gives rise to a surgically correctable form The pheochromocytomas are relatively hypodense or isodense in comparison to the liver on T1
of hypertension 1. weighted spin echo (SE) images (Figure 4) and are highly intense on T2 weighted SE images
(Figure 5). The reason for the difference between the two images is unknown however this may be
due to the water composition in the tissue.
There are 4 stages of pheochromocytoma and they include:
The cause of pheochromocytoma is still unknown. Whilst many of these cases arise sporadically, 25% MRI is considered to be more advantageous in screening for pheochromocytoma due to the
•Localized benign pheochromocytoma – tumor is found in one area and has not spread to other
of pheochromocytomas are inherited from germ line mutations in the RET, VHL, NF1, SDHB, SDHC or superior tissue characterisation capability in conjunction with its multicoplanar abilities greater than
tissue; may not be cancerous
SDHD genes 2. These genes control various factors such as cell cycle progression in the Krebs cycle, that of CT 12.
glucose metabolism and oxygen sensing only to name a few. •Regional pheochromocytoma – cancer has spread to lymph nodes and other tissue around the area
•Metastatic pheochromocytoma – cancer has spread to other parts of the body
•Recurrent pheochromocytoma – cancer has returned after treatment 9 http://www.medicalarts.com.au/media/gallery/129.jpg

•Very uncommon disease

• Can occur at any age, mostly young adults
• Equal chance of occurrence in males and females 3
• Can affect either one or both adrenal glands, or outside the adrenal glands (extra-adrenal)
• Present only in < 1% of hypertensive patients
• Extra-adrenal cases have higher chance of malignancy 4
http://blog.timesunion.com/running/files/2
010/06/heart_rate_monitor.jpg
•Familial / hereditary
1. Multiple endocrine neoplasia (MEN)
2. Von Hippel-Lindau syndrome
• Without any family history (sporadic) 5
Related to sympathetic nervous system hyperactivity:
•Headache
•Excessive sweating
•Elevated heart rate 5
Other symptoms include tremor, nausea, vomiting and fever.
Signs and symptoms usually follow a pattern of high blood pressure
and then low blood pressure. High blood pressure usually begins with
a change in breathing and a pounding or forceful heartbeat 6.
Medical treatment followed by surgical removal of
the lesion is the technique used to treat
pheochromocytomas. Adequate medical treatment is
critical to provide a good outcome from surgery.
Phenoxybenzamine is commonly administered to
patients to increase blood volume and also block
excessive amounts of epinephrine and norepinephrine
secreted by the tumour 13. A beta-blocker is then
prescribed to the patient to control the effects of the
excessive production of adrenaline from the tumor.
http://emedicine.medscape.com/article/379861-overview http://medind.nic.in/ibn/t05/i4/ibnt05i4p433.pdf
The surgical treatment of choice for pheochromocytoma is laparoscopic adrenalectomy. This
Fig. 1 CT slice Fig. 2 Ultrasound image procedure involves the removal of the adrenal glands. Careful planning prior to surgery is
necessary for the most favorable treatment outcome for the lesion 13. Excessive manipulation of the
tumor should be avoided during the surgery to avoid life threatening hypertension.
Many imaging modalities may be performed to determine and diagnose Pheochromocytoma. These
modalities may include that of CT, ultrasound, plain films and MRI. In cases where pheochromocytomas are malignant, chemotherapy or radiation therapy may be
utilised after surgery 14.
The initial imaging findings of a benign and a malignant pheochromocytomas are almost identical, only
with the presence of metastasis would define that a lesion is malignant. The size of a
pheochromocytoma may vary from 1-20 cm 10 and usually seen when they are larger than 3cm 11.
The pheochromocytoma prognosis is dependant on the staging of the pheochromocytoma and the
On CT it appears as round or oval discrete mass with homogenous density. Central necrosis, calcification patient’s age and health condition 9. Benign pheochromocytomas within the adrenal gland have
and cystic changes may be present 10 (Figure 1). excellent outcomes after adrenalectomy. Recurrent, metastatic pheochromocytoma, or tumours that
are unable to be removed surgically have a fair to poor outlook. Patients who are pregnant with
Pheochromocytoma have a poor outcome; where the mortality rate is about 50% for both mother
Ultrasound may also be used to diagnose pheochromocytoma.
pheochromocytoma In ultrasound, the lesions appears
and fetus 15.
hypoechoic with few echogenic areas 10 (Figure 2).