Adrenocortical Carcinoma

What are the Suprarenal glands? What is Adrenocortical Carcinoma? How does Adrenocortical Carcinoma present? How is Adrenocortical Carcinoma detected? What is staging and grading? How is cancer of the Adrenal Cortex treated? What are the side effects of Adrenocortical Cancer treatment? What is the importance of follow up?

What are the Suprarenal glands?

The suprarenal glands or the adrenal glands are two special glands located above each kidney in the back of the upper abdomen. The glands have two layers. The outer one is known as the adrenal cortex, the inner one as the adrenal medulla. The cells in the adrenal cortex are responsible for producing important hormones which are essential for proper body functioning. These hormones also affect male and female sexual characters. The Urinary System with the two Suprarenal Glands on top of the Kidneys

 

 

 

 

 

 

 

 

What is Adrenocortical Carcinoma?

Cancer which originates in the adrenal cortex is known as Adrenocortical Carcinoma. Cancer which originates in the adrenal medulla is known as pheochromocytoma (covered in a separate section).

 

 

 

 

 

 

 

 

 

How does Adrenocortical Carcinoma present?

Adrenocortical Carcinoma presents in a number of peculiar ways and if any of the following symptoms appear and will not go away, please do not hesitate to meet your doctor. The symptoms are: Pain in the abdomen Loss of weight without any particular reason. Extreme weakness. There could be other symptoms which are seen because of high levels of functional hormones secreted by the tumour such as: Weakening of bones or pathological fractures. Diabetes. Masculinization in the form of deepening of the voice. Hirsutism or excessive hair on the face. Swelling of the sex organs. Swelling of the breasts. Cancers of the adrenal cortex usually do not make extra hormones and they are called nonfunctioning tumours. Cancers originating in the adrenal medulla usually produce hormones and therefore they are called functioning tumours.

 

 

 

 

 

 

How is Adrenocortical Carcinoma detected?

If the patient is symptomatic, the doctor will order special blood or urine tests to see whether the hormones or the byproducts of the hormones which are metabolized are present or not. The doctor may also use a computerized tomography scan or a CT scan of your abdomen or special x-rays to find out the presence of the tumour. MRI may also be used.

 

 

 

 

 

 

 

What is staging and grading?

These are the following stages of Adrenocortical Carcinoma: Stage 1. The cancer is less than 5 cm or less than 2 inches and has not spread into tissues adjacent to the adrenal gland. Stage 2. The cancer is greater than 5 cm and has not spread into tissues around the adrenal gland. Stage 3. The cancer has invaded tissues around the adrenal gland or has spread to the lymph nodes next to the adrenal gland. Stage 4. Cancer has spread to tissues or organs in the area and to lymph nodes around the adrenal cortex or has spread to other parts of the body. (Lymph nodes are small, bean-shaped structures which act as stations for receival of a special clear fluid called lymph which traverses the entire body akin to the circulatory system and cancer cells have a predilection of using these lymph pathways to be dispersed all over the body. Therefore cancer cells are usually first lodged in the lymph nodes adjacent to the area which is being drained). Recurrent Adrenocortical Carcinoma is that which has come back or recurred after the primary treatment is over. It may reappear in the adrenal cortex or it may reappear in other parts of the body.

 

 

 

 

 

 

How is cancer of the Adrenal Cortex treated?

A lot depends on the patient's constitution, the grade of his disease and its staging. Patients are usually treated by a team of specialists which utilizes a multipronged approach. This includes an endocrinologist, an oncosurgeon and a radiotherapist. The plan of treatment is tailored to fit a patient's requirements. If necessary, chemotherapy may become an add on. Treatment strategies. Surgery is still the bastion of treatment for carcinoma of the adrenal gland and the surgery to remove the adrenal gland is called adrenalectomy. Tissues adjacent to the gland or lymph nodes adjacent to the gland, draining the adrenal gland may also be removed en bloc along with the main gland which is cancerous. Removal of lymph nodes is known as lymphadenectomy. The alternative local therapy to surgery is radiation therapy. This involves the use of high energy, penetrative rays to destroy cancer cells. It affects cancer cells only in the zone treated. Radiation therapy is also employed for palliation i.e. control of symptoms alone in an advanced cancer. Radiation therapy can also be used in adjunct to surgery or chemotherapy, either before or after. Teletherapy or external radiation is given via a machine remote from the body while brachytherapy or internal radiation is given by implanting a radioactive source within the involved tissues. Patients may or may not require both modalities of radiation. Radiotherapy, after initial planning, usually takes just 5 to 10 minutes per day, 5 days a week for about 6 weeks. This time period is flexible depending upon the modality of radiation used. Besides local therapy, the best attempt to control cancer cells circulating in the body and lodged at places other than the adrenals is by using systemic therapy (therapy which affects the entire systems of the body) which is in the form of injections or oral drugs. This form of treatment is called chemotherapy. This form of treatment is given in a cyclical manner (each set of drugs is repeated usually after every 3-4 weeks). Chemotherapy can also be used in combination with surgery, radiotherapy or both, either before or after. Besides treatment for cancer, a patient may also receive therapy to alleviate symptoms caused by the extra hormone production by the cancer. For stage I Adrenocortical Carcinoma, one of the following options may be entertained: Surgery to excise the entire lesion. For stage II Adrenocortical Carcinoma, one of the following options may be entertained: Surgery to excise the entire lesion. However the results are not as good as in stage I. For stage III Adrenocortical Carcinoma, one of the following options may be entertained: Surgery to excise the entire lesion. However the results are poorer than even stage II. Radiation therapy. Chemotherapy if the tumour size is measurable with CT scan or if the tumour is overproducing hormones. Stage IV Adrenocortical Carcinoma is a pretty advanced disease and one of the following options may be exercised: Chemotherapy for which trials are on to test out new drugs. Radiation to bones where the cancer has spread surgery to remove cancer in places where it is feasible. Removal of metastasis. Recurrent Adrenocortical Carcinoma treatment depends on many factors including where the cancer has come back and what treatment has previously been received. In some cases repeat surgery can be effective in decreasing the symptoms of the spread of the disease by removing some of the tumour. This is called a debulking procedure. It is important to realize that chemotherapy and radiation therapy play a very minimal role in the adrenocortical cancer treatment. Once the tumour has pushed the patient beyond realistic survival chances after surgery, then it is anybody's guess as to the prognosis over the patient.

 

 

 

 

 

 

What are the side effects of Adrenocortical Cancer treatment?

Unfortunately, treatment for cancer does cause damage to healthy tissues as well, the tissue involved being dependent upon the modality of treatment selected. This results in certain side effects. Please ask the clinician about any possible side effects before any form of treatment commence. The effects of radiation depend upon the dose of radiation, the size of the area radiated, and the number and size of each fraction. The commonest side effect is extreme fatigue. Although bed rest is good, most radiotherapists advise the patient to be as mobile as possible. Another common problem is that of radiation dermatitis in which the skin covering the radiated area becomes red, dry, itchy, and may show signs of scaling off. This will slowly settle down after radiation ceases, but there may be a permanent 'bronzing' of the skin. It is important to note here that this skin problem is associated only with teletherapy or external radiation therapy. Radiation also may cause nausea and vomiting, diarrhea, urinary discomfort. There may also be a fall in the white blood cells, which are needed by humans to fight infection. Usually the radiotherapist can suggest the drugs and diet necessary to alleviate such problems. The various drugs in chemotherapy cause a varied spectrum of side effects. The severity and variability of these symptoms are very individualistic, changing from patient to patient. Some of the common side effects are: Infections Skin rashes. Loss of hair. Diarrhea. Vomiting. Tingling and numbness in the fingers and toes. Hearing loss. Most are temporary and recede after therapy is over. Hair growth gradually starts after cessation of chemotherapy. The medical oncologist (chemotherapist) will usually be able to reduce the severity and spectrum of these side effects. The psychology of the patient is very important during therapy. The better psychologically prepared the patient is, the better are his or her chances of having decreased side effects.The patient should drink large quantities of liquids while therapy is in progress, and preferably avoid uncooked or raw food. Water for drinking should be filtered or boiled. Any relative or person who has any infection such as common cold should be asked to desist from coming close to the patient.

 

 

 

 

 

What is the importance of follow up?

The importance of follow-up cannot be overemphasized. It is a fatal fallacy to assume on the patient's part that once the primary or the first phase of treatment is over, then he or she is cured. It must be stressed here that cancer has a notorious proclivity of coming back again and again. The only way to fight this is to pick up any problem when it is still early, so that it can be nipped in the bud. And the only way to do this is to follow up when the doctor calls you for the next visit. Each such visit will include a detailed history, a physical examination (including an internal i.e. a rectal or vaginal examination), a chest x-ray, an ultrasonography or a CT scan, and various other blood studies as indicated.