Table of Contents
- Introduction to Adrenal Cancer
- Grading and Staging
Introduction to Adrenal Cancer
Adrenal glands are a pair of glands resting on the top of each kidney. Their function is to produce hormones that take part in numerous processes occurring in the body.
They are composed of two parts:
- Adrenal Cortex (the outer part): It produces three different types of steroid hormones that are cortisol, aldosterone, and androgens. Cortisol is released in times of stress and has a multitude of effects on the human body. Aldosterone mostly deals with the body’s salt and water balance while androgens are sex hormones produced only in small quantities.
- Adrenal Medulla (the inner core): This part produces hormones called norepinephrine and epinephrine (aka adrenaline) that prepare the body for a fight-or-flight response.
What is Adrenal Cancer?
In the human body, cells continually divide to produce newer ones but this process occurs under strict controls. Cells that fail to respond to bodily signals and continue dividing non-stop give rise to aggregates of cells that were never needed in the first place. These nests of cells hence form masses or lumps inside the organ, commonly called as tumors.
- Cancers occur when such cells get more aggressive, grow very rapidly, and spread to other sites in the body via blood or lymph. Tumors that have this propensity to spread outside the gland are known as malignant tumors.
- The majority of adrenal tumors are non-cancerous or benign, which usually stay within the glands and never invade tissues outside.
Adrenal cancers are also often known as adrenocortical carcinomas (the cancers of the adrenal cortex), as this is the most common type. (Tumors of the adrenal medulla are known as pheochromocytomas). Some of these tumors may produce hormones (functional tumors) while others may not (non-functional tumors).
Luckily, most of the adrenal tumors are non-cancerous, so they do not have serious health implications.
Key statistics regarding Adrenal Cancer:
- Adrenal cancer is quite rare, usually affecting people in their first and fifth decades of life.1
- Women are affected more often than men.1
- Tumors arising in the pediatric population are more likely to be functional while those present in older ones do not usually produce hormones.1
- Most cases of adrenal gland tumors are adrenocortical carcinomas.2
Symptoms of Adrenal Cancer
Tumor cells may retain the ability to produce hormones and hence such tumors are called functional tumors. They produce the very hormone that was originally produced by their parent cell, for example, tumors arising in the region having cortisol producing cells will produce cortisol and not aldosterone if it is functional. Other tumors might not produce any hormone and thus are non-functional.
Most adrenal cancers arise from the adrenal cortex and are called adrenocortical carcinomas. A minor proportion may also belong to the adrenal medullary cells and such tumors are called malignant pheochromocytomas or neuroblastomas.2 In the majority of the population, the symptoms occur due to the overproduction of hormones.3 Non-functional tumors, on the other hand, only come to notice, when they have grown large enough to press upon surrounding structures, causing either pain or a feeling of heaviness in the abdomen (belly).4
Cortisol is the hormone more likely to be overproduced, but in some patients, cortisol and sex hormones may be equally high.5
Adrenal cancers may occasionally come to notice on imaging done for some other purposes.5
Depending upon the part in which the tumor is present, you may feel variable symptoms. Some common ones include:
- Weight gain, especially around abdomen and face
- Purple stretch marks on the abdomen
- Muscle weakness
- Nausea and vomiting
- High blood sugar levels
- High blood pressure
- Easy bruising
In children, symptoms like overproduction of sex hormones (androgens) make it easier to detect adrenal cancer much earlier than adults, for example, growth of excessive hair due to male hormones or early development of breasts because of female hormones. While in adults, if the tumor is producing hormones of the opposite gender, only then early detection is a possibility.
It is important to note that all the symptoms listed above are very much likely to be caused by some other condition as well. So if you notice any, consult with your health care provider who might recommend further investigations to rule out the possibility of cancer.
Causes and Risk Factors
Cancers occur when DNA in cells undergoes some abnormal changes (mutations). These changes can be inherited or acquired during life. Exposure to harmful radiations or substances can increase the risk of mutations.
Although very little data is available about the causes of adrenal cancers, research has highlighted the presence of genetic predisposition.5 Such familial cases mostly appear in childhood and are associated with the presence of certain familial syndromes.5
Other studies have tried finding a relationship between smoking tobacco and adrenal cancers,6 but further studies are needed to validate this idea.
Diagnosis of Adrenal Cancer
Your doctor will take your complete medical history and perform the necessary physical examination. They may also run the following tests to confirm the diagnosis:
- Blood and urine tests: These are performed to check if the levels of certain hormones in the body are normal.
- CT scan: Imaging tests can locate cancer and determine its level of spread. A CT scan can be used to view abnormal growth in adrenal glands.7
- MRI: It can also be used to establish the diagnosis.
- PET scan (positron emission tomography): This scan can provide a clear picture of the tumor as well as its metastases in the body. 8
- Laparoscopy: In this, a thin, bendable tube with a small video camera attached to its far end is used to locate cancer and assess its spread. It is inserted into the patient’s abdomen through a small incision. It can be used as a mode of treatment too.
- Biopsy: Needle biopsy usually guided by imaging could be done and the sample is further sent to a laboratory. Some doctors may not ask for it as it carries some risk of spreading the tumor.
Spread of cancers from other areas of the body to the adrenal gland is much more common than cancer primarily originating there, it is important to keep this information in mind when making a diagnosis.9
Grading and Staging of Adrenal Cancer
It is a clinical criterion used to evaluate the aggressiveness and spread of cancer. The doctor identifies the stage on the basis of imaging tests or the biopsy report.
Adrenal cancers lie anywhere from stage I – IV, and the higher the stage, the more aggressive the tumor is. These stages are based on the size of the tumor and its spread to nearby organs, lymph nodes and/or distant metastases.
Treatment of Adrenal Cancer
The treatment options for adrenal cancers include surgery, radiotherapy, and chemotherapy. With advanced techniques, the stay at the hospital has been shortened given no complications occur.
- Adrenalectomy: In this procedure, the whole of the affected gland is removed. Since there is a pair present, removing one does not cause serious problems. The surgeon may also want to resect some lymph nodes and tissue present around if there is a suspicion of the spread of cancer to these areas.
- Chemotherapy and Radiotherapy: Some patients may need sessions of chemotherapy (drugs that kill cancer cells) or radiotherapy (radiation beams like X-rays, directed at cancer cells to kill them) to kill any cancer cells that might have remained after surgery or if cancer returns. Higher stage cancers that have already metastasized to distant locations can only get symptomatic benefits such as lowering of pain by these methods.
- Laparoscopy: Open adrenalectomy is the widely used option for the removal of the gland but in recent times, techniques like laparoscopy are also under discussion.
In laparoscopy, several small incisions are made and a fine tube is inserted in the abdomen through one of these incisions. Instruments required to perform the surgery can be inserted through other incisions and the tumor has to be resected by breaking it into smaller parts. The main advantage of using this method is that it avoids a large incision so the healing of wounds is faster. But it is mainly reserved for smaller or non-cancerous tumors 10 as it involves breaking the mass in order to remove it, which may cause it to spread.
You can decide with your doctor as to which option or combination of options is best suited for you depending upon the stage of your cancer. Surgeries that are believed to have a better prognosis are usually preferred.
- Wooten, M. D., & King, D. K. (1993). Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer, 72(11), 3145-3155.
- Hsing, A. W., Nam, J. M., Co Chien, H. T., McLaughlin, J. K., & Fraumeni Jr, J. F. (1996). Risk factors for adrenal cancer: an exploratory study. International journal of cancer, 65(4), 432-436.
- Luton, J. P., Cerdas, S., Billaud, L., Thomas, G., Guilhaume, B., Bertagna, X., … & Bonnin, A. (1990). Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. New England Journal of Medicine, 322(17), 1195-1201.
- Hutter Jr, A. M., & Kayhoe, D. E. (1966). Adrenal cortical carcinoma: clinical features of 138 patients. The American journal of medicine, 41(4), 572-580.
- Else, T., Kim, A. C., Sabolch, A., Raymond, V. M., Kandathil, A., Caoili, E. M., … & Hammer, G. D. (2014). Adrenocortical carcinoma. Endocrine reviews, 35(2), 282-326.
- Chow, W. H., Hsing, A. W., McLaughlin, J. K., & Fraumeni, J. F. (1996). Smoking and adrenal cancer mortality among United States veterans. Cancer Epidemiology and Prevention Biomarkers, 5(2), 79-80.
- Čtvrtlík, F., Heřman, M., Študent, V., Tichá, V., & Minařík, J. (2009). Differential diagnosis of incidentally detected adrenal masses revealed on routine abdominal CT. European journal of radiology, 69(2), 243-252.
- Yun, M., Kim, W., Alnafisi, N., Lacorte, L., Jang, S., & Alavi, A. (2001). 18F-FDG PET in characterizing adrenal lesions detected on CT or MRI. Journal of Nuclear Medicine, 42(12), 1795-1799.
- Uberoi, J., & Munver, R. (2009). Surgical management of metastases to the adrenal gland: open, laparoscopic, and ablative approaches. Current urology reports, 10(1), 67.
- Gumbs, A. A., & Gagner, M. (2006). Laparoscopic adrenalectomy. Best practice & research Clinical endocrinology & metabolism, 20(3), 483-499.