I've Just Been Diagnosed. with Kidney Cancer: What s Next?

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1 I've Just Been Diagnosed with Cancer: What s Next?

2 Table of Contents Introduction Message from a Survivor What are the s and What is Their Function? What is Cancer? What are the Types of Cancer? What are the Known Risk Factors and Genes Associated with the Development of Cancer? Genes and the Risk of Cancer What are the Symptoms of Cancer? What Tests are Available to Detect the Presence of Cancer and Determine if it has Spread? Stages of Cancer Surgical Options for Cancer Surveillance Post-Nephrectomy Additional Forms of Treatment for Cancer Chemotherapy Molecular Targeted Therapy Biologic Therapy Radiation Therapy Clinical Research Glossary

3 Introduction T his brochure will help you get through the first hours and days after learning that you have kidney cancer. It contains basic information that will help you ask the questions you may need to ask and start thinking about what to do next. The brochure will direct you to reliable information about kidney cancer and current ways to manage the disease. Who We Are Copyright 2014 by Alamo City Cancer Council Author: Sharon T. Wilks, M.D., F.A.C.P. The Alamo City Cancer Council (ACCC) is a non-profit organization based in San Antonio, Texas. It works with the representatives from organizations in Bexar County that focus on needs of individuals afflicted with cancer. The ACCC wishes to thank all of those individuals who support this organization and its mission in helping educate those who are diagnosed with cancer. Please visit the ACCC website: Message from a survivor I am a cancer survivor. This is my story of how I survived cancer and how I m moving on strong. It all started in January I thought I was dealing with Cedar Fever (allergies). I had been coughing a lot, running a fever and was very tired. I was prescribed some antibiotics but it did not resolve. Then I developed a low hemoglobin count and I was transfused with two units of blood. During this treatment, my liver enzymes were high so an ultrasound of the stomach was done to check for gallstones. When the test results came in, I was told it showed a tumor on my right kidney. I then had a CT scan which confirmed that it looked like I had cancer. I was seen by a urologist who felt that the cancer did not look like it had spread. I was told that I would need an operation and my surgeon assured me that he was going to check everything and make sure that everything was good. After I was told the C word that I had cancer, my mind began to wander and I started to think of the possibility that it had spread and that I was going to die. How long do I have? Do I draw up a will? So I said to myself, Bull! You re going to overcome this! Just put your faith in God! I also told myself that I am not done here yet on this planet! There is still more for me to do! I underwent my surgery and I was clear. A few weeks later I was good to go back to work, which was awesome. I was told that I do have a risk of it coming back and I agreed to participate in a clinical trial. I have always been into comic books about super heroes and all the great feats they perform and with the study, [I feel] this is my chance to be a hero. If we can prove that through this study that the drug can help prevent cancer from coming back, that would be great! It s not a cure, but a means to prevention, which is a great thing! I have survived kidney cancer with the help of my family, friends, doctors, nurses and my faith. If I can be a hero, so can you! Don t let the criminal that is cancer win! BE STRONG! BE BRAVE! SURVIVE! WIN! Be a hero for YOURSELF and your family! FIGHT! Jaime San Antonio, Texas 2 3

4 What are the s and What is Their Function? The kidneys are two bean shaped organs that are located in the upper back of the abdominal cavity and their job is to filter the blood to remove excess water, salt and waste products. This process of filtering leads to urine production. Urine collects in a portion of the kidney known as the renal pelvis (see Figure 1). The renal pelvis connects to the bladder via a tube known as the ureter. Eventually, urine is eliminated from the body through another tube that is referred to the urethra. Dysfunction can lead to kidney failure, requiring external support for the body, like dialysis. Most functions related to the kidney can be handled by one kidney but the demand of waste elimination is best provided by both kidneys. Another important organ connected to the kidney is the adrenal gland which is located on top of the kidney and is responsible for production of androgens. What are Androgens? Androgens are steroid hormones critical in stress metabolism, male sexual organ development and have a role as a source for estrogen production in older women. What is Cancer? This is a cancer that forms in the kidneys or renal pelvis and accounts for only about 2-3% of all adult cancers, making it the seventh most common cancer in men and the ninth most common cancer in women. Cancers of the kidney refer to malignant tumors that have the potential of invading and damaging nearby organs and tissues. These cells can spread to other distant organs through the blood stream or regional lymphatics. This distant spread is referred to as metastasis. These metastases if formed will carry the same type of cell and behavior found in the kidney. That means that even though it's far from the kidney it should be referred to as metastatic kidney cancer to bone, lung, etc. Thus, if cancer cells spread, treatment that works for kidney cancer also controls cancer growth that has spread from the kidney to a distant area. Common organs that kidney cancer spreads to are the lungs, bone and liver. 4 Right Ureters Bladder What are the Types of Cancer? Renal Cell Cancer There are several types of kidney cancer: the most common type is referred to as renal cell cancer (80-85% of all kidney cancers). Subtypes of renal cell cancer include: clear cell renal cell cancer papillary renal cell carcinoma chromophobe renal cell carcinoma collecting duct renal cell carcinoma unclassified renal cell carcinoma Inferior Vena Cava Adrenal Gland Figure 1 Renal System Descending Aorta Renal Artery (red) & Vein (Blue) Left (in cross-section) Pelvis of 5

5 Transitional cell carcinoma This is another type of cancer that usually affects the renal pelvis. This cancer is similar to cancers with the same tissue type in the bladder and treatments applied to this form of kidney cancer follow the treatment approaches used for bladder cancer. Wilm s tumor Also called a nephroblastoma, it is the most common type of childhood kidney cancer. Renal Sarcoma Renal sarcoma is a rare type of kidney cancer that starts in the kidney s connective tissue or blood vessels. What are the Known Risk Factors and Genes Associated with the Development of Cancer? Most people who are diagnosed with kidney cancer have no identifiable risk factors. However these are some known factors: Suggested factors include: Smoking - cigarette smoking for long periods of time has been highly associated with development of kidney cancer. Obesity High Blood Pressure/Hypertension Chronic Dialysis Von Hippel-Landau (VHL) is a rare disease that occurs in some families. It is caused by inheritance of the VHL gene. Individuals with this disease also can be troubled with development of cyst/tumors of the eyes, brain and other areas of the body. Males - more men are diagnosed with kidney cancer than women. Genes and the Risk of Cancer Inheritance of the VHL gene increases the risk of kidney cancer. What are the Symptoms of Cancer? Most individuals with early forms of kidney cancer have no symptoms. Hematuria blood in the urine is one of the most common signs of this disease. Patients may also have back or flank pain due to the pressure of the tumor growth in the lower pelvis or they may have unexplained weight loss or recurrent fever. 6 What Tests are Available to Evaluate the Presence of Cancer and if it has Spread? Urinalysis - testing the urine for increased red blood cells that can only be detected under the microscope or detection of cancer cells in the urine (cytology analysis) Blood tests these tests may show that the kidney function (creatinine or BUN elevation) may be impaired. Intravenous Pyelogram (IVP) - this is a procedure where an injection of a dye is administered to a patient into the vein in the arm. This dye travels through the patient's blood stream and collects into the kidneys. The dye or contrast can be seen on an x- ray which can reveal a growth or change in the kidney or renal pelvis or ureter. Ultrasound - this is a special test that uses sound waves which create a picture of the kidney and can show a solid tumor or cyst if present in the kidney. CAT/CT scan - this is an x-ray that uses a computer to produce pictures/x-rays of the internal organs including the kidney and nearby tissue and other organs like the lung and liver. Bone Scan this is a type of x-ray that uses a tracer that is injected into the patients' vein to determine if the bone has any cancer cells or metastases. Biopsy of the kidney - this procedure involves removal of a sample of tissue from the kidney to look for cancer cells. In order to be sure the biopsy is done of a growth in the kidney, a specialist who performs this may require an ultrasound or CT scan to guide them where to place the biopsy needle. Stages of Cancer It is important for selection of treatments to know what the extent or stage the kidney cancer is. This process of evaluation allows the team of doctors to know if the tumor is confined to the kidney or urinary tract system or if it has spread to nearby or distant areas of the body. 7

6 Stage I The kidney tumor measures up to 7 cm (two &3/4 inches); the tumor is confined to the kidney. Figure 2 Adrenal Gland Stage III Tumor involves the nearby lymphatics/lymph node or Tumor invades into but not beyond adrenal gland or the layers of fat and fibrous tissue that surrounds the kidney. Tumor may involve the lymph node or The tumor cells have spread to a nearby large blood vessel (renal vein or the inferior vena cava) Multiple lymph node metastasis Figure 4 Renal Artery Tumor is 7cm or smaller Cortex Ureter Lymph nodes Tumor Stage II The tumor is larger than 7 cm but is still confined to the kidney. Adrenal Gland Figure 3 Adrenal Gland Perinephric fat Tumor Tumor is larger than 7 cm Cortex Renal Artery Ureter Renal Vein tumor Ureter 8 9

7 Stage IV Tumor extends beyond the fibrous tissue (Gerota's fascia) that surrounds the kidney or Cancer cells are involving more than one lymph node group or Cancer has spread beyond the local region to a distant site like the lung, bone or liver. Brain Lung Liver Figure 5 Surgical Options for Cancer Surgery is the most common form of treatment and this procedure is usually carried out by a specialist known as an Urologist or Urologic Oncologist. The best candidates for surgery are those who have early stages of kidney cancer. Radical Nephrectomy The surgeon removes the entire kidney along with the adrenal gland and some tissue around the kidney known as Gerota's fascia. Nearby regional lymph nodes may also be removed. Most doctors would consider this more extensive type of surgery for those who have: Tumors that are large (greater than 7 cm) and are located in the center of the kidney Suspected lymph node involvement Found the tumor to be attached to the renal vein or inferior vena cava or if there is evidence of tumor involving the adrenal gland also. This procedure can be done with an open incision or via laparoscopy. Figure 6 Bone Adrenal Gland Tumor Ureter 10 11

8 Simple Nephrectomy The surgeon removes only the kidney. Partial Nephrectomy The surgeon removes only the portion of the kidney that the tumor exists in. Typically, this kind of surgery is done if a patient has a small kidney cancer < 7 cm and only has one kidney or if the tumor involves both kidneys and to avoid permanent kidney failure and dialysis, the unaffected portion of the kidney is not removed. Laparoscopic nephrectomy This operation is done through several small incisions instead of one large incision. Special long instruments are inserted through the incisions made. One of these tubes is a laparoscope which has a small video camera at the end of this tube. This device allows the surgeon to see the tissues and assist with his/her ability to remove the kidney. Often the surgeon uses a morcellator, a surgical instrument used for division and removal of large masses. This tool allows the surgeon to cut into and grasp the tumor which is pulled into the hollow cylinder of the morcellator. This procedure is as effective as an open incision approach with a shorter recovery required and generally less pain after surgery. This option is less likely to be offered to patients with large tumors or tumors that involve the renal vein or have spread to the regional lymph nodes. Figure 7 Other forms of surgery for kidney cancer that limits removal of normal kidney tissue: Arterial Embolization This treatment is done typically to avoid surgery and help someone have relief of symptoms related to the kidney cancer like pain or excess bleeding. A specialist in radiology is often involved in this procedure which involves insertion of a narrow tube or catheter in the large artery of the leg and this tube is advanced up to the major blood vessel known as the renal artery. Once the artery is located, the specialist injects a dye or material that will lead to blockage of the blood flow to the kidney that has the cancer present. This blockade allows the cancer to die as it does not received oxygen or other critical substances that it normally gets from the blood flow. Cryotherapy Cryotherapy is use of cold therapy (cold gases) that is delivered to the tumor via a needle that is inserted into the tumor-this process kills the tumor but spares nearby tissues. Radiofrequency or Thermal Ablation This procedure involves the use of high-energy radio waves to heat the tumor via use of a specialized probe. This can be with minimal harm and as an outpatient. Enucleation This is a procedure that allows only the removal of the kidney tumor without dissection into the portion of the kidney that does not contain tumor. Surveillance Post-Nephrectomy The optimal time frame for post-operative surveillance has not been established but most individuals undergo some type of routine imaging using CT scans most often about every 6-12 months post surgery for about 5 years post-diagnosis

9 Additional Forms of Treatments for Cancer Chemotherapy for Cancer Chemotherapy uses anti-cancer drugs that can be given into the vein (intravenous) or in pill form (oral therapy). Most conventional chemotherapy drugs like Vinblastine, 5- Flourouracil/Capecitabine, Gemcitabine have limited effectiveness in this disease. Molecular Targeted Therapy Most successful therapies are those referred to as targeted therapy based upon the molecular and genetic changes in the kidney cancer. Several drugs that have a focused mechanism of action for kidney cancers have been approved in recent years by the FDA. These drugs are referred to as targeted therapy and are known to work on specific areas that influence kidney cancers to grow including angiogenesis (growth of new blood vessels) and key pathways at the molecular level: 2 common targets or pathways include the Vascular Endothelial Growth Factor (VEGF) and mtor pathways. These drugs usually lead to some cancer control and are typically used when the kidney cancer has spread or recurred and local measures are not an option for care. Commonly used Tyrosine Kinase Inhibitors include Sunitinib [Sutent ], Axitinib [Inlyta ], Sorafenib [Nexavar ], & Pazopanib [Votrient ]. One commonly used Angiogenesis Inhibitor in cancer is Bevacizumab [Avastin ]. mtor Inhibitors These are drugs that affect the mtor pathway which is located inside the tumor cell and is part of a pathway known as the Phosphoinositide 3-Kinase- [PI3Kinases pathway] and the AKT pathway. This pathway is regulated by PTEN tumor suppressor genes. This pathway, when mutations occur in the PTEN pathway, is well known to many doctors as a strong signaling pathway that influences tumor cell growth and metastases. New drugs have been made available that block the signaling message via these pathways and are known as mtor inhibitors. Temsirolimus and Everolimus [Affinitor ] are examples of mtor inhibitors. 14 Biologic Therapy Biologic therapy also is referred to as immunotherapy. The aim of this therapy is to boost the patient's immune system to fight cancer growth. The most common immune therapies include Interleukin-2 (IL-2) and Interferon-alpha. Both of these agents referred to as cytokines are used to shrink the kidney cancer. These therapies, particularly IL-2, have proven to be very effective methods for longterm tumor control. Both of these drugs cause significant side effects so careful selection of individuals for this therapy is recommended. Radiation Therapy Rarely radiation therapy, which uses high energy rays to kill cancer, has been used. cancers are known to be fairly resistant to radiation but in cases where the cancer has metastasized to the bones, radiation therapy may be helpful to relieve pain. Clinical Research New therapies are being evaluated often for patients with kidney cancer. It is important to ask about clinical trials and if they may be eligible to participate. Clinical trials are carefully controlled research studies that are done with patients who volunteer for them. To participate in clinical trials, certain tests may be run to determine if a patient is eligible. Many new options of care are provided for patients with kidney cancer through clinical trial and this may be an avenue for a patient to receive state of the art cancer treatment. 15

10 Glossary Adrenal gland - this is a structure that is located on top of the kidneys-they secrete hormones that are released in response to stress. Biologic therapy - considered a form of immunotherapy that uses some method, including drugs, that enhances, induces, stimulates or suppresses an immune response. Immunotherapy is used in management of many cancers to help the immune system attack and destroy cancer cells. Bladder - this is an organ in the genitourinary tract that collects urine that is formed and excreted by the kidneys. It is described as a hollow muscular organ and is located in the pelvis. Urine enters the bladder via the ureters. Chemotherapy - this is a type of treatment that uses medicines/drugs to induce cancer cell death. Many drugs in this class target different parts of cell division and stop mitosis from continuing. Technically, any drug that stops tumor cells from growing is considered a type of chemotherapy. Clinical research - this is a type of study that evaluates the safety and effectiveness of medicines, devices, diagnostic tools that are being evaluated for human use. Embolization - this is considered a non-surgical procedure typically performed by doctors who are known as interventional radiologists. The procedure involves the selective blockage (occlusion) of blood vessels. This process leads to stoppage of blood flow to an organ and induces death to that tissue/organ. Gerota's fascia - this is a layer of connective tissue that encapsulates (surrounds) the kidney. Growth factors - this is a naturally occurring substance in the body that has the ability to stimulate cell growth and cell maturation. It is usually a protein or steroid hormone that can circulate to distant areas of the body. These substances stimulate or signal messages between cells. Ionizing radiation - this is a type of radiation that uses particles of energy for cell damage. This type of radiation is a high energy source of radiation. It is invisible and not able to be detected by human senses. Molecularly targeted therapy - this is a frequently used therapy that uses medications to block the growth of cancer cells by specifically targeting molecules needed for tumor growth. These therapies have proven to often be more effective and less harmful to non-targeted cells and tissues. PTEN gene - Phosphatase and tensin homolog (PTEN) is a protein that is encoded by a gene. PTEN enzyme functions normally to stop signals that tell cells to divide and grow. Typically, if this gene is functioning properly, cells undergo programmed cell death (apoptosis). Mutation or loss of this gene appears to be critical in the formation of human cancers. This is a gene known to be a suppressor gene and when it mutates, this can lead to significant cancer growth. Radiation therapy - this is a type of cancer treatment that uses certain concentrations of ionizing radiation that is used to control or kill malignant cells. Signaling - this is a process that allows a message to be passed on in a pathway from the outside or inside of a cell. This message usually leads to activation of proteins and or genes that leads to a specific response within the cell. Tumor cells use signaling to communicate messages within key paths that stimulate nuclear activation and cellular growth and proliferation. Tyrosine kinase inhibitors - these are types of targeted therapies which are enzymes that block the action of ore or more protein kinases. These kinases/enzymes usually generate energy within the cell by adding a phosphate group to a protein. These enzymes usually generate messages for cellular growth and these inhibitors are specifically designed to prevent these messages for growth and proliferation within the cell. Ureter - this is a tube that is made up of smooth muscle and connects the kidneys to the bladder and propels urine from the kidney to the bladders. Urethra -this is a tube that connects the bladder to the genitals for removal of fluids from the body. Urinalysis - this is a test that analyzes changes in the urine. It allows for an assessment of cells, protein, blood and bacteria that may be present in the urine

11 Contributions are Needed and Appreciated If you have found this information helpful, we would like you to consider giving a donation to the ACCC to help with continuance of the organization's mission to educate our public about aspects related to cancer. We provide materials like this daily to patients and their families, nurses and physicians in order to help with the explanation of what might come next after a diagnosis of cancer. Your kind gift will help continue this special mission of education. The ACCC feels strongly that education and information is empowering and provides hope for tomorrow for problems we face today. Contributions can be sent to the Alamo City Cancer Council, 100 NE Loop 410, Suite 600, San Antonio, TX or click the Make a Donation link at More Information on Cancer American Society of Clinical Oncology (ASCO) patient website: The National Cancer Institute: The American Cancer Society: The National Foundation: Cancer Association: