indiacancer.org - Pheochromocytoma Cancer

		What are the Suprarenal Glands?
		What is Pheochromocytoma?
		How does Pheochromocytoma present?
		How is Pheochromocytoma detected?
		What is staging and grading?
		How is Pheochromocytoma 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 Pheochromocytoma?

Cancer which originates in the adrenal medulla is known as pheochromocytoma. The cells of origin are the chromaffin cells of the adrenal medulla. It is a rare form of cancer. Usually, only one adrenal gland is involved. Sometimes, though rarely so, it may originate from other tissues such as the urinary bladder or the heart.

Cancer which originates in the adrenal cortex is known as adrenocortical carcinoma.

	How does Pheochromocytoma present?

	Pheochromocytoma 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 called catecholamines secreted by the tumor such as:
		High blood pressure.
		Palpitations.
		Headaches.
		Heart problems.
		Stroke.
	Cancers originating in the adrenal medulla usually produce hormones and therefore they are called functioning tumors. Pheochromocytoma may be part of a syndrome (a group of unrelated diseases or symptoms which occur together) called MEN (Multiple Endocrine Neoplasia), where it may be associated with thyroid or parathyroid tumors. This condition shows a familial trait, that is, it tends to run in families.

	How is Pheochromocytoma 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 terms with which you must become familiar, if you are dealing with any form of cancer.

When we stage a cancer, we try to establish carefully degree of spread of the cancer and if indeed there is spread, to what extent and involving which organs.

The grading of a cancer is a microscopic issue, in which the pathologist lets you know how aggressive this particular type of cancer is ; well differentiated means less aggressive, moderately differentiated intermediately aggressive and poorly differentiated more aggressive.

Both grading and staging help your clinician to establish the prognosis or how serious your disease is and what is going to be the likely outcome.

Staging may require additional imaging tests such as CT Scan, MRI, Sonography of the abdomen, IVU, Bone scan or chest X-ray.

The stages for pheochromocytoma are:

Localized benign pheochromocytoma. Tumor in only one area without spread. Most pheochromocytomas are like this.

Regional pheochromocytoma. Spread to lymph nodes of the area or adjacent tissues. Lymph nodes are small bean-shaped organs located in groups in various parts of the body, such as in the neck, the armpits, the groin and inside the abdomen, and the central part of the chest. They act as drainage stations for lymph, a clear transparent fluid which acts as a transporting medium for infection fighting cells as well as cancer cells.

Metastatic pheochromocytoma. Disease has spread to different parts of the body.

Recurrent pheochromocytoma is that which has come back or recurred after the primary treatment is over. It may reappear in the adrenal medulla or it may reappear in other parts of the body.

	How is Pheochromocytoma 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 pheochromocytoma and the surgery to remove the adrenal gland is called adrenalectomy. One or both the adrenals may have to be removed. Adjacent involved tissues or lymph nodes may also have to be sacrificed. 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 localized benign pheochromocytoma, one of the following options may be entertained:
		Unilateral or bilateral adrenalectomy.
	For regional pheochromocytoma, one of the following options may be entertained:
		Unilateral or bilateral adrenalectomy, with removal of as much disease as is possible.
		If cancer remains after surgery, drugs to control high blood pressure.
		Teletherapy (rarely given, as rarely helpful).
		Chemotherapy.
	For metastatic pheochromocytoma, one of the following options may be entertained:
		A debulking procedure, in which as much of the tumor as is possible is removed.
		Drugs are given for alleviation of blood pressure problems.
		Teletherapy to reduce symptoms.
		Chemotherapy.
	For recurrent pheochromocytoma, one of the following options may be entertained:
		A debulking procedure, in which as much of the tumor as is possible is removed.
		Drugs are given for alleviation of blood pressure problems.
		Teletherapy to reduce symptoms.
		Chemotherapy.

	What are the side effects of Adrenocortical cancer treatment?

	Unfortunately treatment for cancer does cause damage to the healthy tissues. Please ask the clinician about any possible side effects before any form of treatment commences. 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:
		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 will usually be able to reduce the severity and spectrum of these side effects. The psychology of the patient is very important during therapy. Better psychologically prepared the patient is, better are his chances of having decreased side effects. The patient should drink large quantities of water while the therapy is in progress, and preferably avoid uncooked or raw food. Water should be filtered or boiled. Any relative or person who has any sort of 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 followup 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.