ONCOLOGY DISTURBANCES IN IMMUNOLOGY AND INFLAMMATORY I. OVERVIEW OF IMMUNOLOGIC SYSTEM A. BASIC STRUCTURES 1.

Lymphoid System consists of the lymph nodes, spleen, thymus, tonsils, lymphoid tissues and bone marrow (involves in blood cell formation). Thymus Gland assists in T lymphocyte formation which is involved in cell mediated immunity. Bone marrow sources iliac crest, sternum and bone cavities throughout the body. serve as diagnostic predictor for immunologic, hematologic and oncologic disorders. give rise to B lymphocytes and humorally mediated responses (humoral immunity) involve production of antibodies. Tonsils found in palatine area of the oropharynx in the mouth. Lymph Nodes found throughout the body and consist of small rounded mass of tissue from which the lymph fluid drains. Mucosa associated lymph tissue consists of grouping of lymph tissue that is found in many organs of the body that work together to promote an immune response. Spleen composed of white and red pulp (white pulp - composed of B and T lymphocytes; red pulp composed of erythrocytes) site of destruction of RBC as well as storage site of blood filters and removes foreign materials, worn out cells and forms of cellular debris. Mononuclear Phagocyte System (MPS) protect body by participating in the immune response; secretes chemical component and factors (enzymes, complement protein and interleukins). monocytes are the largest components of WBC macrophages are considered to be the matured cells of MPS migrate to different areas of the body specialized cell to perform their function of defense. 2. Normal Immune Response a. Defense the body provides for a communication network of protection that involves both specific and non-specific forms of defense. Non-specific defenses external reaction that include anatomic and chemical barriers ( skin, mucous membrane, sweat and sebaceous glands, acidic secretions and bodys ph ) Non-selective activated against any foreign substance that the body would encounter. Specific defenses internal physiological reactions of the body that include both cell mediated and humorally mediated antibodies (considered specific unique substances that required activation). the body initiates its immune response in the presence of ANTIGEN (a protein substance that triggers antibody production). b. Homeostasis the body seeks to maintain an immune balance where it can successfully removed damaged cells. there is balanced response of circulation and resident lymphocytes to maintain adequate protection. c. Surveillance the ability of the body to use memory and recognition in order to maintain adequate protection. the body will remember the activation response even if the person doesnt remember the specific insult. 3. Types of Immunity a. Acquired Immunity Active Acquired Immunity is a long term response in an organism that leads to the development of antibodies that offer protection. developing antibodies in response to having the disease process or by a response to artificial antigens ( vaccine or toxoid ). this immunization response can be boosted and maintained via repeated injection. Passive Acquired Immunity requires that antibody be introduced to the individual, either by maternal transfer (placenta/colostrums) or immune serum antibody injection to promote a specific antigen response.

b. Natural Immunity the type of immunity that exist in an individual is related to a specific race or genetic traits. the individual is born with natural immunity. c. Humoral Immunity this involves the recognition of antigens by the B lymphocytes B lymphocytes memory cells lead to a more rapid response by remembering the original insult. plasma cells secrete IMMUNOGLOBULINS (a group of lycoproteins) Types of Immunoglobulins 1. IgG makes up of the total immunoglobulins the only one that crosses the placenta located at plasma, interstitial fluid protects against viruses, bacteria and toxins complement fixation, secondary immune response 2. IgA 2nd most common immunoglobulins in the bloodstream present in the body secretions, tears, saliva, colostrums and breastmilk first line of defense against organisms invading the respiratory or GI or urinary tract. lines mucous membrane, protects body surfaces. 3. IgD located in the bloodstream (plasma) in a very small amount present on lymphocytes may increase in myeloma or with some CNS tumor 4. IgE present in the plasma interstitial fluid and exocrine secretions increase in allergic/anaphylactic states and in the event of parasitic infestations 5. IgM most important component in primary immune response found in plasma IgM antibodies are indicators of an active infection Involves in ABO antigens Complement fixation d. Cell Mediated Immunity T lymphocytes recognize a specific major histocompatibility complex (MHC) a group of proteins that play a role in auto immune recognition and tissue rejection on the surface of T cells help to define specific function receptor sites. CD means cluster of differentiation CD markers serve as an important prognostic indicator of immune function and are used in the diagnosis of HIV and AIDS. e. Other Immune System Participants Natural Killer Cell activity is present at birth, increases as one reaches adulthood. Cytokins lymphokins and monokins (infernos, interleukins mediators of cellular immunity, promote development of cytotic T cells. Complement System group of glycoproteins that are activated in sequential order and provide a link of humoral response. II. ALTERED IMMUNE RESPONSE: HYPERSENSITIVOTY REACTION

1. Hypersensitivity an abnormal exaggerated response to a specific indicator that leads to an

overactive immune response. 2. Cell and Coombs Classifications of Hypersensitivity Reaction Type I: Anaphylactoid Reactions Class: Immediate Hypersensitivity Immunity: Humoral Etiology: Involves the characteristic activation of IgE Histamine is released. Clinical Signs/Symptoms: Bronchospasm, wheezing, rhinorrhea, urticaria to angioedema. Management: Antihistamine, decongestants, corticosteroids, Epinephrine Nursing Care: Immediately withdraw the offending allergen; manage the client according to ABC protocol Type II: Cytotoxic and Cytolytic Reaction Class: Activation of complement Immunity: Humoral Etiology: Involves the production of autoantibodies that results in destruction of ones own cells or tissues.

Clinical Signs/Symptoms:

Management: Nursing Care:

- hemolytic reactions (transfusion, erythroblastosis fetalis, hemolytic anemia and drug-induced hemolysis) to target cells; - destructions seen in Good Pasteurs Syndrome (an autoimmune disease affecting pulmonary and renal systems) proper identification during BT, awareness of potential drug interactions can cause antigen complex activation remain with the client for 15 minutes during BT, proper verification of blood (inc. registration #).

Type III: Immune Complex Reactions involves the production of antigen-antibody complexes (binding together of an antibody and an antigen). leads to the activation of serum factors, causing inflammation and leading to activation of complement cascade. rheumatoid arthritis and systemic lupus erythematosus are examples of type III reactions Etiology: complement activation impacts vulnerable organs and leads to intravascular changes. Clinical Manifestations: - Arthrus reaction involves a localized inflammatory response with excess IgG causing joint pain, pyrexia, lymphadenopathy - Serum sickness involves a systems response edema and necrotic tissue. Diagnostic/Lab. Findings: - complement assay indicates acute or chronic. - ESR is elevated - Proteinuria on urinalysis Management: - analgesics, antihistamines and topical steroids (provide symptom relief) - disease process is self-limiting because the use of human ATS and the availability of the antibiotics. Nursing Care: - be aware that it is possible for localized inflammatory reactions to develop at the site of serum injections after one week; can be followed by a more systematic response involving regional and generalized lymphadenopathies. - if symptoms arise, monitor client for potential complications (organ damage can occur, kidney can be compromised). Type IV: Delayed Hypersensitivity Reactions a form of cell mediated immunity involving T lymphocytes involves the recognition and response of T lymphocytes to foreign substances Clinical Manifestations: - there is a wide range of presentation from tuberculin response, poison ivy, and contact dermatitis to transplant or graft rejection (pyrexia, pain, edema and failure of transplanted organ) - edema, ischemia and eventual tissue destruction may ensue. Diagnostic/Lab. Findings: - purified protein derivative ( PPD ) test result of induration > 5 mm identifies type IV hypersensitivity to TB - abnormal test results indicating declining function of the transplanted organ are used to diagnose transplanted rejection. Management: - monitor for evidence of potential transplant rejection - medicate patient with immunosuppressive protocol drugs to prevent tissue rejection. - identify potential irritants that can cause contact dermatitis and avoid exposure. - use topical and oral medication to alleviate many of the symptoms complained. AUTOIMMUNITY Autoimmunity is an abnormal response of the bodys immune system whereby it perceives self as a threat. There are several mechanisms of action that can affect the autoimmune process Cell mediated immunity (associated with an abnormal T-cell response); there can be an overabundance of t-cytotoxic (killer) cells or deficiency of t-suppressor (helper) cells. Antibody mediated autoimmunity (associated with the development of autoantibodies that affect specific receptor sites causing tissue and organ damage), e.g. Graves Disease and Myasthenia Gravis. Immune Complex Disease (associated with the deposition of immune complexes at the serum level). Genetic Components: Genetic traits are associated with autoimmune diseases. Human leukocytes antigens (HLAs) genetic markers found on chromosomes 6, are involved with the diagnosis of many auto immune diseases and also used for tissue typing. Treatment for Autoimmunity

Immunosuppressive agents and corticosteroids are given to suppress the abnormal immune
response. Symptoms management: anti-inflammatory agent (to minimize pain) Plasmapheresis is used to remove circulating immune complexes (plasma is removed from the body, sent through a machine membrane that traps immune complexes, and is returned to the body). Progression of Disease Autoimmune diseases are characterized by acute exacerbation of a chronic condition. They can be seen across the life span, affecting both children and adult. In many cases, splenectomy has been performed as part of therapeutic management of many autoimmune diseases. Current therapeutic regimen: chemotherapy AUTOIMMUNE DISEASES 1. Systemic Lupus Erythematosus 2. Rheumatoid Arthritis 3. Myasthenia Gravis 4. Scleroderma: Progressive Systematic Sclerosis A multisystem disease present with fibrosis (hardening) of visceral organs and the skin. The resultant fibrosis leads to inability of involved organs to function with normal motility. 5. Rheumatic Heart Disease The patient develops antibodies which react to the bodys own antigens in the connective tissues of the heart, as well as in other systems. The antibodies are produced in response to repeated and untreated streptococcal infections, usually of the throat. A late sequelae of acute rheumatic fever (an inflammatory process and is not contagious or infectious). Fever, malaise, weakness, anorexia, abdominal pain, weight loss Major manifestations: - carditis, polyarthritis, chorea, erythema marginatum Diagnostic Examinations : - Increase ESR, WBC, will be reactive to protein tests; indicating presence of inflammation. - Antistreptolysin O (ASO) titer; antistreptokinase (ASK) titer test for strep antibodies. - Throat Culture : + for group A beta hemolytic streptococcus - ECG reveals prolonged PR interval Management: - Drug therapy, rest, nutrition and care of specific symptoms. - Antibiotics (Penicillin), corticosteroids for inflammation. - Careful handling of patient for joint pain. A. PRIMARY IMMUNODEFICIENCY DISORDER Are caused by primary defect or deficiency involving B lymphocytes, T lymphocytes, complement or phagocytic cells that result in severe infection that can be recurrent or chronic in nature. Manifestations: - clients overall immune response is abnormal, leading to infections may be caused by opportunistic agents (opportunistic infections) - recurrent infection can lead to subsequent tissue and organ damage - signs and symptoms of infection and inflammation: fever, chills, cough, respiratory complaints associated with difficulty of swallowing and breathing. Management: - infection prophylaxis, early treatment of infection and replacement of immunologic factors. - bone marrow transplant or thymus transplant - Medication therapy: antimicrobial, antifungal, gamma globulins to support and maintain deficient immunoglobulins. B. SECONDARY IMMUNODEFICIENCY DISORDER Disease process cause a secondary immunosuppressive response DM, burns, malnutrition, some autoimmune disease and AIDS are examples of precipitating disease process. C. HIV (Human Immunodeficiency Syndrome) An RNA retrovirus attacks the immune system at the CD4 antigen, causing cell mutation that leads to eventual disease progression. D. AIDS A progression of HIV Disease

Diagnosed when there is a CD4 count of 200/min in the presence of an AIDS defining disease (opportunistic infections, malignancies, recurrent pneumonia)

DISTURBANCES IN CELLULAR FUNCTIONING OR CELLULAR ABBERATIONS TERMINOLOGY: Neoplasia development of an abnormal type of growth that is unresponsive to normal growth mechanisms Neoplasm a group of clump of neoplastic cells, synonymous with tumor Metastasis the ability of the cancer cells to disseminate and establish growth in another area of the body at distance from its origin Invasion occurs when cancer cells infiltrate adjacent tissue surrounding the neoplasm Aberrant cellular growth an alteration in normal cellular growth, which usually means that the normal cell escape the hosts control of growth and differentiation Hyperplasia refers to an increase in number of normal cell Metaplasia refers to conversion from a normal pattern of differentiation of one type of cell into another type for that tissue Dysplasia refers to alteration in the shape, size, appearance and distribution of cells. Anaplasia refers to disorganized, irregular cells that have no structure and have loss differentiation. The result is always malignant. CANCER - comes from the Greek word Karkinos: meaning a crab-a graphic description of disease growth pattern - new growth of tissue resulting from a continuous proliferation of abnormal cells that have the ability to invade and destroy other tissues. Symptoms / early warning signs: C Change in bowel or bladder habits A A sore throat that does not heal U Unusual bleeding or discharge T Thickening or lump in the breast or elsewhere I Indigestion or difficulty in swallowing O Obvious change in a wart or mole N Nagging cough or hoarseness U Unexplained anemia S Sudden unexplained weight loss Any of these symptoms: any unexplained lump, pain, weight loss, or lethargy, should be referred to a doctor. Although one or more symptoms may be indicative of something other than cancer, a check-up to confirm their cause is the best course of action. EPIDEMIOLOGY 1. Viruses and Bacteria Viruses are thought to incorporate in the genetic structure of cells. Thus, altering the future generations of cell population-perhaps leading to a cancer a virus at some point infected the cell causing genetic damage to the cells deoxyribonucleic acid (DNA) thus leading to the development of cancer Example: Epstein-Barr Virus highly suspected as a cause in Burkitts lymphoma, nasopharyngeal cancer, and some type of non-hodgkins lymphoma and Hodgkins disease Herpes simplex virus type II, Cytomegalovirus and human papillomavirus type 16, 18, 31 are associated with dysplasia and cancer of the cervix The hepatitis B virus is implicated in cancer of the liver. The human T cell lymphotropic virus may be a cause of some lymphocytic leukemias and lymphomas The human immunodeficiency virus is associated with Kaposis sarcoma Bacterium Helicobacter pylori has been associated with an increased incidence of gastric malignancy 2. Physical agents Exposure to sunlight or radiation, chronic irritation or inflammation and tobacco use Excessive exposure to ultraviolet rays of the sun, especially in fair skinned, blue or green -eyed people increases the risk of skin cancers More than 80% of exposure to radiation is from natural sources Ionizing radiation from cosmic rays and radioactive materials such as radon gas, radium and uranium About 15% of radiation exposure comes from diagnostic or therapeutic procedures Radiographs, radiation therapy, and radioisotopes used in diagnostic imaging 5% of all secondary cancers are clearly linked to radiation therapy from a previous cancer

3. Chemical agents About 75%of all cancers are thought to be related to the environment Tobacco has been linked not only with lung cancer but also with oropharyngeal, bladder, pancreatic, cervical, and kidney cancer People can be exposed to chemicals in the workplace have proven to be carcinogens or cocarcinogens o Aromatic amines and anliline dyes o Pesticides and formaldehydes o Betel nut and lime, cadmium, chromium compounds o Arsenic soot, and tars, asbestos, benzene o Most hazardous chemicals produce their toxic effect by altering DNA structure in body o The liver, lungs and kidney are the organ systems most often affected presumably because of their roles in detoxifying chemicals 4. Genetic and Familial Factors Abnormal chromosomal patterns of cancer have associated with extra chromosomes, too few chromosomes or translocated chromosomes Specific cancers with underlying genetic abnormalities chronic myelogenous leukemia, meningiomas, acute leukemias, retinoblastomas, Wilms tumor and skin cancer, including malignant melanoma Approximately 5% to 10% of cancers of adulthood and childhood display a familial predisposition- premenopausal breast cancer In cancers wit familial predisposition, individuals may develop multiple cancer, commonly, two or more first degree relatives share the same cancer type Cancers associated with familial inheritance includes retinoblastomas, pheochromocytomas, malignant neurofibromatosis, and breast, ovarian, endometrial, colorectal, stomach, prostate and lung cancers 5. Dietary Factors Diet is probably linked to about 30 to 35% of all environmental cancers. Dietary substances can be proactive (protective) carcinogenic or cocarcinogenic. Foods associated with increased cancer risk includes: fats, alcohol, salt-cured or smoked meats, foods containing nitrates and nitrites and a high caloric dietary intake Foods that appear to reduce cancer risk include high-fiber foods, cruciferous vegetables(cabbage, broccoli, cauliflower, Brussels sprouts, kohlrabi) carotenoids (carrots, tomatoes, spinach, apricots, peaches, dark green and deep yellow vegetables possibly vitamins E, C, zinc and selenium Obesity is associated with endometrial cancer and possibly postmenopausal breast cancer, also increase the risk for cancers of the colon, kidney and gallbladder 6. Hormonal agents Tumor growth may be promoted by disturbance in hormonal balance either by the bodys own (endogenous) hormone production by administration of exogenous hormones Cancers of the breast, prostate and uterus are thought to depend on endogenous hormonal levels for growth Oral contraceptives and prolonged estrogen therapy are associated with increased incidence of hepatocellular, endometrial and breast cancers THE CELL CYCLE The cell replication cycle is divided into the following intervals, or steps with the letter G standing for gap, the interval separating cell division or mitosis (M) and synthesis(S). G0 Phase this phase is the interval in which the cell is at rest for cell division until a trigger in the intermediate environment signal the beginning of the G1 phase. Some cells do not replicate, or they replicate so infrequently that they are said to always be in G0 or resting phase. G1 Phase this phase is the interval in which ribonucleic acid (RNA) and protein are synthesized. S Phase synthesis of both DNA and proteins of new chromosomes occurs during the S phase. The interval of time is probably 6 to8 hours, although it vies in certain cell populations and under different conditions. G2 phase biochemical processes, including the synthesis of some RNA, occur in preparation for mitosis. Little is known about this phase which lasts only a few hours. M phase actual division of the cells (mitosis) occurs during this phase, producing two daughter cells usually ranges from less than an hour to few hours. CHARACTERISTICS MITOTIC CELL DIVISION NORMAL CELLS Mitotic cell division leads to two daughter cells. CANCER CELLS Mitosis leads to multiple daughter cells that may or may not resemble the parent

APPEARANCE GROWTH PATTERN

Cells of some type homogenous in size, shape and growth, regular pattern of expansion. Have characteristic pattern of organization. Do not invade adjacent tissue. Proliferate in response to specific stimuli. Growth in ideal conditions (e.g. nutrients, oxygen, space, correct biochemical environment).

multiple mitotic spindles. Cells larger and grow more rapidly than normal (pleomorphic) i.e. heterogenous in size and shape. Cells have irregular patterns of expansion. Invade adjacent tissues. Proliferation without stimulus. Growth in adverse condition such as lack of nutrients. Do not exhibit contact inhibition. Cell birth exceeds cell death. Loss of cell control as result of cell membrane changes. Growth rate erratic. Able to break off cells that migrate through bloodstream or lymphatics, or seed to distant sites and grow in other sites. Serve no useful purpose. Do not contribute to the well being of the host; parasitic, actually feed off host without contributing anything. If cells functions at all, they do not function normally or may actually cause damage (e.g. lung cancer secretes ACTH and cause excessive stimulation of adrenal cortex). Invade, erode and spread. Invade in the presence of necrosis and inflammatory cells such as lymphocytes.

Have specific, designated purpose. Contributions to the well being of the host. Cells function in specific predetermined manners (e.g. cells in the thyroid secrete thyroid hormone) Cannot invade, erode or spread. Cannot grow in the presence of necrosis or inflammation

FUNCTION

OTHER

CARCINOGENESIS - the process through which normal cells are transformed into malignant or cancer cells. 4 stages of carcinogenesis: 1. Initiation occurs when a carcinogens damages DNA. Carcinogens cause changes in the structure and function of the cell at the genetic or molecular level. This damage maybe reversible or may led to genetic mutations if not repaired; however, the mutations may not lead immediately to cancer. 2. Promotion occur with additional assaults to the cells resulting in further genetic damage 3. At some point, these genetic events results in malignant conversion 4. With progression, the cells are increasingly malignant in appearance and behavior and develop into an invasive cancer with metatasis to distantbody parts. CLASSIFICATION OF NEOPLASMS A. Benign tumors are characterized by entirely localized growth and are usually separated from neighboring tissue by a surrounding capsule. generally grow slowly, and in structure closely resemble the tissue of origin. in some instances they may endanger the patient by obstructing, compressing, or displacing neighboring structures, as in the brain. comes from the latin benigumus (kind) Most common Benign Neoplasms: 1. Fibromas - (fibrosarcoma-malignant) - may grow anywhere in the body, but they frequently make their home in the uterus - are easily removed surgically 2. Lipomas (liposarcoma- malignant) - very common in benign neoplasm, arises in adipose tissue - rarely pressure on surrounding tissues as they expand 3. Leimyomas - a benign neoplasm of smooth muscle origin is the most common benign tumor in women - may develop anywhere in the body, but most often they grow I the uterus rarely these neoplasms become malignant B. Malignant tumors has the ability to spread beyond the site of origin cancerous cells that may invade neighboring tissues by direct extension or infiltration, or by metastasis 1. Carcinoma

- tumor that arises from epithelial cells, the name of the cancer identifies the location(basal cell carcinoma). 2. Sarcoma - tumor arising from supportive tissue, the name of the cancer identifies the tissue affected (osteosarcoma). GROWTH OF THE PRIMARY MALIGNANT TUMOR Environmental Factors Chemicals Radiation Viruses Changes in genome of somatic cells Genetic Factors

Activation of growth promoting gene (oncogene)

Inactivation of growth inhibiting gene (cancer suppressing gene)

Expression of altered products, loss of regulatory gene products.

Malignant Neoplasms CHARACTERISTICS Spread of growth BENIGN NEOPLASM Grows slowly Usually continue to grow throughout life unless surgically removed. May have periods of remissions. Grows by enlarging and expanding. Always remains localized, never infiltrates surrounding tissues. Almost always contained within the fibrous capsule. Capsule does not prevent expansion of the neoplasm but does not prevent growth by infiltration. Encapsulated tumor can be removed surgically. Usually cell differentiated Mitotic figures absent or scanty Mature cells Anaplastic cells absent. Cells function poorly in comparison with normal cells from which they arise. Recurrence extremely when surgically removed. unusual MALIGNANT NEOPLASM Usually grow rapidly. Tends to grow relentlessly throughout life. Rarely neoplasm may regress spontaneously. Grows by infiltrating surrounding tissues. May remain localized (in situ) but usually infiltrates other tissues. Never contained within a capsule. Absence of capsule allows neoplastic cells to invade surrounding tissues. Surgical removal of tumor difficult.

Mode of growth

Capsule

Cells characteristics

Recurrence Metastasis Effects of Neoplasm

Metastasis never occurs. Not harmful to host unless located in area where it compresses tissues or obstruct vital organs. Does not produce cachexia (weight loss, debilitation, anemia, weakness and wasting).

Usually poorly differentiated Cells tend to be anaplastic i.e. young embryonic type. Cells too abnormal to perform any physiologic functions. Occasionally a malignant tumor arising in glandular tissues secretes hormone. recurrence common following surgery because tumor cells spread into surrounding tissues. Metastasis very common. Always harmful to host. Cause death unless removed surgically or destroyed by radiation or chemotherapy. Causes disfigurement, disrupted organ function, nutritional imbalances. May result ulcerations, sepsis, perforation, hemorrhage, sloughing of tissues. Almost always produces cachexia which leaves person to pneumonia, anemia, etc. Poor prognosis if cells are poorly differentiated and evidence of

Prognosis

Very good. Tumor generally

removed

surgically.

metastasis spread exists.

Three Representative Examples of Malignant Neoplasm 1. Carcinoma in situ This is the neoplasm of epithelial tissue that remains confined to the site of origin. In situ carcinoma, it typically affects the cervix. localized and can be removed surgically but can become invasive, eroding into surrounding tissues. 2. Malignant Fibrosarcoma These tumors are similar to benign fibromas, tend to grow in the same sites, may originate as benign fibromas, later becoming malignant. These bulky, well differentiated tumor masses are usually responsive to surgery. Fortunately they rarely metastasize. 3. Bronchogenic carcinoma These tumors account for 90% of all cases of lung cancer. It usually develops in the trachea and lower bronchi. Surgical excision of the tumor is the intervention of choice. This type of cancer readily gives rise to metastasis, if this occurs, surgery is contraindicated. TISSUE OF ORIGIN Almost all names for neoplasms end in the suffix -oma meaning, tumor. For example: An adenomyoma is benign neoplasm that contains both glandular and muscle (Greek genitive: myos) cells. Benign tumors of epithelial origin are classified according to either their microscopic appearance (e.g. adenoma) or their macroscopic appearance (e.g. poly, from the Greek polys for many, and pous for foot) A malignant neoplasm that arises from epithelial tissue is called a carcinoma; (adenocarcinoma). A malignant neoplasm that arises from mesenchymal origins (e.g. blood vessels, lymphatic tissue, nerve tissue) is called sarcoma (from Greek sarx, flesh) Hematopoetic erythrocytes (erythroleukemia) lymphatic tissue (hodgkins disease) plasma cells (multiple myeloma). TUMOR STAGING AND GRADING 1. STAGING Determines the size of the tumor and the existence of metastasis Stage 0: Carcinoma in situ Stage I: Tumor limited to the tissue of origin; localized tumor growth. Stage II: Limited local spread. Stage III: Extensive local and regional spread. Stage IV: Metastasis 2. GRADING Refers to the classification of the tumor cells. Grading system seek to define the type of tissue from which the tumor originated and the degree to which the tumor cells retain the functional and histologic characteristics of the tissue of origin. Samples of cells to be used to establish the grade of a tumor or maybe obtained through cytology (examination of cells from tissue scrapping, body fluids, secretions or washings), biopsy or surgical excision. Grade I: Cells differ slightly from normal cells and are well differentiated (mild dysplasia). Grade II: Cells are more abnormal and are moderately differentiated (moderate dysplasia). Grade III: Cells are very abnormal and are poorly differentiated (severe dysplasia). Grade IV: Cells are immature (anaplasia) and undifferentiated; cell of origin is difficult to determine The TNM system is frequently used: T refers to the extent of the primary tumor (size). T0 no involvement Tis tumor in situ T1, T2, T3, T4 progressive degrees of tumor size and involvement N refers to lymph node involvement N0 abnormal lymph nodes detected N1a, N2a, N3a regional lymph nodes involvement N1b, N2b, N3b regional lymph nodes involvement with metastasis suspected M refers to metastasis M0 no metastasis

M1, M2, M3 indicates ascending degrees of distant metastasis and includes distant lymph nodes

METASTATIC MECHANISM Lymph and blood are key mechanisms by which cancer cells spread. Angionesis, a mechanism by which the tumor cells ensured a blood supply, is another important process. 1. Lymphatic Spread The most common mechanism of metastasis is the lymphatic spread, through the lymphatic circulation. Tumor emboli enter the lymphatic channels by way of the interstitial fluid. Malignant cells also may penetrate lymphatic vessels by invasion. After entering the lymphatic circulation, malignant cells either lodge in the lymph nodes or pass between lymphatic and venous circulation. Breast tumors frequently metastasize in this manner through axillary, clavicular and thorax lymph channel. 2. Hematogenous Spread Malignant cells are disseminated through the blood stream. Hematogenous spread is directly related to the vascularity of the tumor. Few malignant cells can survive the turbulence of arterial circulation, insufficient oxygenation or destruction by the bodys immune system. 3. Angiogenesis Malignant cells also have the ability to induce the growth of new capillaries from the host tissue to meet their needs for nutrients and oxygen. It is through this vascular network that tumor emboli can enter the systematic circulation and travel to distant sites. Large tumors emboli that become trapped in the microcirculation of distant sites may further metastasize to other sites. TUMOR MARKER protein substance found in the blood or body fluids, released either by the tumor itself or by the body as a defense in response to the tumor (e.g. AFP alpha feto protein). MANAGEMENT OF CANCER 1. SURGERY A. Diagnostic Surgery such as biopsy is usually performed in obtaining a tissue sample for analysis of cells suspected to be malignant. Most Common Biopsy a. Excision Biopsy - used for easily accessible tumors of the skin, breast, upper and lower GIT and URT. - this approach not only provides the pathologist what stage and grade the cells with the entire tissue specimen but also decrease the chance of seeding the tumor. b. Incision Biopsy - is performed if the tumor is too large to be removed. In this case a wedge of tissue from the tumor is removed for analysis. - the cells of the tissue wedge must be representative of the tumor mass so that the pathologist can provide an accurate diagnosis. If the specimen does not contain representative tissue and cells, negative biopsy result do not guarantee the absence of cancer. c. Needle Biopsy - performed to sample, suspicious masses that are easily accessible, such as some growth in the breast, thyroid, lung, liver and kidney. B. Surgery as a Primary Treatment the goal is to remove the entire tumor as a much as is feasible (procedure sometimes called debulking) and any involved surrounding tissue, including regional lymph nodes. C. Prophylactic Surgery involves removing non-vital tissues or organs that are likely to develop cancer. The following factors are considered when electing prophylactic surgery. Family history and genetic predisposition. Presence or absence of symptoms. Ability to detect cancer at an early stage. Patients acceptance of the postoperative outcome. e.g. colectomy, mastectomy, oophorectomy D. Palliative Surgery when cure is not possible, the goals of treatment are to make the patient as comfortable as possible and to promote a satisfying and productive life for as long as possible.

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E. Reconstructive Surgery may follow curative or radical surgery and is carried out in an attempt to improve function or obtain a more desirable cosmetics effect. 2. RADIATION THERAPY Ionizing radiation is used to interrupt cellular growth, kill a tumor, and reduce tumor size. Causes lethal injury to DNA, so it can destroy rapidly multiplying cells, as well as normal cells. Types of Radiation Therapy: A. External Beam Radiation Therapy (teletherapy) is the delivery of radiation from a source placed at some distance from the target site. It is administered in the RT department by high energy X-Ray or gamma X-Ray machines (e.g. Linear accelerator, cobalt betratron, or a machine containing a radioisotope). The major advantage of high energy radiation is its skin- sparing effect that is the maximum effect of radiation occurs at tumor depth in the body and not on the skin surface. The radiation oncologist makes specific locations for radiation treatment using semi-permanent type of ink. Treatment is usually given 15-30 minutes per day, 5 days per week, for 2-7 weeks. The client does not pose risk for radiation exposure to other people. Side Effects: a. tissue damage to target area (erythema, sloughing, hemorrhage). b. ulceration of oral mucous membrane c. GI nausea, vomiting, diarrhea d. fatigue e alopecia f. radiation pneumonia g. immunosuppression Client Education: a. wash marked area of the skin with plain water and pat skin dry, no soaps, deodorants, lotion powder on site during the duration of treatment. b. avoiding rubbing, scratching or scrubbing the treatment site, do not apply extreme heat or cold. c. wear soft, loose fitting clothing over the treatment area. d. protect skin from sun exposure during the treatment and for at least 1 year after the treatment is complete ( Sunblock SPF15 ). e. maintain proper rest, diet are essential to promote health and repair of normal tissues. Nursing Care: a. monitor for adverse side effects of radiation b. monitor for significant decrease in WBC and platelet counts. B. Internal Radiation Therapy involves placement of specially prepared radioisotopes directly into or near the tumor itself (brachytherapy) or into the systemic circulation. Types of IRT Sealed Source RT in which radioactive material is administered in a container. Unsealed Source RT in which the radioactive material is administered systematically, such as by injection or orally. RADIATION SAFETY STANDARDS 1. Distance the greater distance from the radiation source, the lesser the exposure dose of ionizing rays. Distance and radiation exposure are inversely related, thus the intensity of radiation decrease inversely with the square of the distance from the source. For Example: If you stand 4 feet from the source of radiation, you are exposed approximately the amount of radiation you would receive at 2 feet. 1 meter 2 meters (1/4 of exposure) 3 meters 4 meters 1/16 exposure 2. Time - you should aim to minimize the amount of time you are exposed to the radiation source, although you must still meet the clients care needs. Your exposure time should generally be limited to 30 minutes of direct care per 8 hour shift. 3. Shielding the use of shielding devices whenever possible reduces radiation exposure. The dose of X-Rays and gamma rays reduces as the thickness of the lead shield is increased. SIDE EFFECTS: a. fatigue b. anorexia c. immunosuppresion CLIENT EDUCATION: a. avoid close contact with others until treatment is completed. b. maintain balance diet, small, frequent meals. c. fluid intake 2 3 L/day d. if implant is temporarily, maintain bed rest to avoid dislodging the implant.

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e. excreted body fluid may be radioactive, double flush toilets after use. NURSING MANAGEMENT: a. protect from exposure to radiation: time, distance and shielding. b. place client in private room c. instruct visitors to maintain at least a distance of 6 feet from the client and limit visits to 10 30 minutes. d. ensure proper handling and disposal of body fluids, assuring the container are marked appropriately. e. in the vent of dislodged implant, use long handed forceps and place the implant into a lead container, never directly touch the implant. f. no pregnant women to come in contact with radiation sources. 3. CHEMOTHERAPY Involves the administration of cytotoxic medications and chemicals to promote tumor cells death. The IV route is the most preferred for administration of chemotherapeutic agents. They may also be administered oral, intrathecal, topical, intra-arterial and intracavity. Is a systematic intervention and appropriate when: disease is widespread the risk of undetectable disease is high the tumor cannot be resected and is resistant to radiation therapy. Goals of Chemotherapy: a. cure b. control c. palliation of manifestations Types of Chemotherapy: 1. Adjuvant Chemotherapy - the client who is at risk for the recurrence but shows no evidence of current disease may be the candidate to this type of therapy. 2. Neoadjuvant Chemotherapy refers to the preoperative use of chemotherapy to reduce the bulk and lower the stage of a tumor, making it amenable to surgery or possibly even cure with subsequent local therapy. Chemotherapy disrupts the cell cycle in various phases interfering with cellular metabolism and reproduction. According to the cell kill hypothesis, during each cell cycle, a fixed percentage of cells are killed by chemotherapy leaving some tumor cells remaining. These necessities repeated dosage of chemotherapy is in order to reduce the number of cells, allowing the bodys immune system to destroy the remaining tumor cell. Chemotherapeutic Agents: 1. Alkalyting agents non-phase specific and act by interfering with the DNA replication (Cytoxan, Busulfan). 2. Anti-metabolites interferes with metabolites nucleic acids necessary for RNA and DNA synthesis (5 Fluoroucil, Methotrexate) 3. Cytotoxic antibiotics disrupt or inhibit DNA synthesis 4. Plant alkaloids Vinka alkaloids phase specific, inhibiting cell division. Side Effects: 1. Bone marrow suppression a. decreased WBC count (immunosuppression) risk for infection, avoid crowds, people with infection meticulous personal hygiene, avoid undercooked and raw fruits and vegetables no pets, no visitors with infection b. decreased platelet count (thrombocytopenia) monitor stool and urine for bleeding (>20,000 platelet count) assess skin of ecchymosis, petechiae and trauma for shaving, use electric razor only avoid contact sports and other activities that may cause trauma avoid dental work or other invasive procedure (IM injection) 2. GI effects: anorexia, nausea and vomiting, diarrhea 3. Stomatitis inflammation of the mouth use of soft toothbrush, mouth swabs during acute episode avoid mouthwash containing alcohol use chloroxidine, mouthwash to decrease risk to hemorrhage and protect gum

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 for xerostomia apply lubricating agent to protect the mucous membrane, use artificial
saline, hard candy or mints to help with dryness avoid smoking and alcohol drink cool liquids, avoid hot and irritating foods

4. Alopecia encourage patient to choose a wig before hair loss occurs in order to match texture and hair color care of hair and scalp (no blow dryer) allow client to express feelings concerning altered body image 5. Fatigue assure client that it is a normal response to chemotherapy and it does not indicate progression of disease. allow rest period in between Expected outcome of clients with Cellular Disorders: 5 year survival rate for cancer is 59%. Cancer of the breast, colon, rectum, cervix, prostate, oral cavity, skin 5 year survival rate is 80%. Approximately 552,000 cancer related deaths occurred in 2000. DETECTION AND DIAGNOSIS Physical Examination - involves inspection and palpation of all accessible sites, especially the skin, neck, breasts, abdomen, testicles, and lymph-node areas Screening and Self-Examination Cervical Smear can detect cervical cancer. It can prevent this cancer because it detects pre-cancerous cells. Bowel Cancer Screening - one method under consideration uses a thin flexible tube (a sigmoidoscope) to detect polyps in the bowel that, if left, may turn cancerous. Breast Cancer Screening through breast self examination and mammography. Mammography - is a special X-ray technique that is used to visualize soft tissues of the breast as a means for screening women for breast cancer. Biopsy - the only definitive method for the diagnosis of a cancer. In a biopsy, a section of tissue is removed from the tumor itself or from a metastasis. Steps in controlling cancer: 1. Do not smoke. 2. Avoid sunburn. 3 Take up offers of cancer screening tests. 4. Eat a healthy diet. 5. Drink only moderate amounts of alcohol. 6. Observe safety rules in jobs where exposure to chemicals, radiation, and other hazards increases risk. TREATMENT 1.Surgery The principal approach to curing cancer is to remove all the malignant cells by a surgical operation. In the past this meant the removal of all of the involved tissue and as much potentially involved tissue as possible, including adjacent tissues and lymph node. 2.Radiation Therapy Radioisotope cobalt-60 is used as the source of gamma radiation. A high dose of this radiation is guided by laser targeting to a localized area of treatment. 3.Chemotherapy Chemotherapy is the use of drugs in the treatment of cancer. 4.Hormone Therapy Tissues that are hormone-dependent, such as the breast, prostate, endometrium (uterine lining), and thyroid, are responsive to hormone manipulation. This may consist of removing the source of the stimulating hormone or the administration of various hormones, antihormones, and hormone blockers, such as tamoxifen. Other approaches: Involves biological agents that stimulate certain cells, which can then attack the malignant cells. The best example is the use of interleukin-2 to stimulate the patients lymphokine-activated killer lymphocytes (LAK cells). Other New Approaches gene therapy Employs various methods to introduce genetic material into the cancer to make it more recognizable to the immune system.

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ONCO-SPECIFIC DISORDERS LEUKEMIA Description Leukemia is a malignant exacerbation in the number of leukocytes, usually at an immature stage, in the bone marrow. Leukemia may be acute, with a sudden onset and short duration, or chronic, with a slow onset and persistent symptoms over a period of years. Leukemia affects the bone marrow, causing anemia, leucopenia, the production of the immature cells, thrombocytopenia, and a decline in immunity. The cause is unknown and appears to involve gene damage of cells, leading to the transformation of cells from a normal state to a malignant state. Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications. Classification of Leukemia Acute Lymphocytic Leukemia Mostly lymphoblasts present in bone marrow. Age of onset is less than 15 years. Chronic Lymphocytic Leukemia Mostly lymphocytes present in the bone marrow. Age of onset is after 50 years. Acute Myelogenous Leukemia Mostly myeloblasts present in the bone marrow. Age of onset is between 15 and 39 years. Chronic Myelogenous Leukemia Mostly granulocytes present in the bone marrow. Age of onset is after 50 years.

Mouth Care for the Client with Mucositis Inspect mouth daily. Offer complete mouth care before and after every meal and at bedtime. Brush teeth and tongue with a soft-bristled toothbrush or sponges. Provide mouth rinses every 12 hours (saline or sodium bicarbonate and water, as prescribed). Administer topical anesthetic agents to the mouth sores as prescribed. Avoid the use of alcohol- or glycerin-based mouthwashes or swabs. Avoid foods that are hard or spicy.

NON-HODGKINS LYMPHOMA Description Non-Hodgkin's lymphoma (NHL) is cancer of the cells of the lymphatic system. In nonHodgkin's lymphoma, cells in the lymphatic system either divide or grow without order or control, or old cells do not die as cells normally do. Non-Hodgkin's lymphoma can start almost anywhere in the body. It may occur in a single lymph node, a group of lymph nodes, or an organ such as the spleen. Non-Hodgkin's lymphoma can spread to almost any part of the body, including the liver, bone marrow, and spleen. Over time, lymphoma cells replace the normal cells in the bone marrow. This causes bleeding problems and infections. As the lymphoma cells spread, the body becomes less and less able to produce blood cells that carry oxygen to other tissues or to protect itself from infection.

HODGKINS DISEASE Description Hodgkins disease (lymphoma) is a malignancy of the lymph nodes that originates in a single lymph node or a single chain of nodes. Metastasis occurs to other, adjacent lymph structures and eventually invades nonlymphoid tissue. The disease usually involves lymph nodes, tonsils, spleen, and bone marrow and is characterized by the presence of Reed-Sternberg cell in the nodes. Possible causes include viral infections and previous exposure to alkylating chemical agents.

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 The prognosis depends on the stage of the disease; the prognosis is excellent in children with localized disease. The primary treatment modalities are radiation and chemotherapy; each may be used alone or in combination, depending on the clinical staging of the disease. Bone marrow transplantation may be consideration in treating Hodgkins disease. Staging in Hodgkins Disease STAGE I Involvement of a simple lymph node region or an extralymphatic organ or site. STAGE II Involvement of two or more lymph node regions on the same side of the diaphragm or localized involvement of an extralymphatic organ or site. STAGE III Involvement of lymph node regions on both sides of the diaphragm. STAGE IV Diffuse or disseminated involvement of one or more extralymphatic organs with or without associated lymph node involvement. BRAIN TUMORS Description An infratentorial (below the tentorium cerebelli) tumor is located in the posterior third of the brain ( primarily in the cerebellum or brainstem ) and accounts for the frequency of symptoms resulting from increased intracranial pressure (ICP). A supratentorial tumor is located within the anterior two thirds of the brain, mainly the cerebrum. The signs and symptoms of a brain tumor depend on its anatomical location and size and to some extent on the age of the child. Therapeutic management includes surgery, radiation, and chemotherapy; the treatment of choice is total removal of the tumor without residual neurological damage. Positioning Following Craniotomy Assess the physicians order for positioning, including the degree of the neck flexion. If a large tumor was removed; the child is not placed on the operative side because the brain may shift suddenly to that cavity. In an infratentorial procedure the child usually is positioned flat and on either. In a supratentorial procedure the head usually is elevated above the heart level to facilitate cerebrospinal fluid drainage and to decrease excessive blood flow to the brain to prevent hemorrhage. Never place the child in the Trendelenburgs position because it increases ICP and the risk for hemorrhage.

WILMS TUMOR (NEPHROBLASTOMA) Description Wilms Tumor is a tumor of the kidney that may present unilaterally and localized or bilaterally, sometimes with metastasis to other organs. The peak incidence is at 3 years of age. The occurrence is associated with genetic inheritance and with several congenital anomalies. Therapeutic management includes a combined treatment of surgery (partial to total nephrectomy) and chemotherapy with or without radiation, depending on the clinical stage and histologic pattern.

PANCREATIC CANCER Description Pancreatic cancer is the most common neoplasm affecting the pancreas. Pancreatic cancer is more common in blacks than in whites, in smokers, and in men. The occurrence of pancreatic cancer has been linked to diabetes mellitus, alcohol use, history of previous pancreatitis, smoking, ingestion of a high-fat diet, and exposure to environmental chemicals. Symptoms usually do not occur until the tumor is large; therefore the prognosis is poor. GASTRIC CANCER Description Gastric cancer is a malignant growth in the stomach. Risk factors include a diet high in complex carbohydrates, grains, and salt, and low in fresh, green, leafy vegetables and fresh fruit; smoking; alcohol ingestion; the use of nitrates and a history of gastric ulcers. Complications include hemorrhage, obstruction, metastasis, and dumping syndrome. The goal of treatment is to remove the tumor and provide a nutritional program. PROSTATE CANCER

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Description This slow-growing cancer of the prostate gland is usually an androgendependent type of adenocarcinoma. The risk increases in men with each decade after age 50. Prostate cancer can spread via direct invasion of surrounding tissues or by metastasis, through the bloodstream and lymphatics, to the bony pelvis and spine. Bone metastasis is a concern. INTESTINAL TUMORS Description Intestinal tumors are malignant lesions that develop in the cells lining the bowel wall or develop as polyps in the colon or rectum. Complications include bowel perforation with peritonitis, abscess and fistula formation, hemorrhage, and complete intestinal obstruction. Metastasis occurs via the circulatory or lymphatic system or by direct extension to other areas in the colon or other organs. CERVICAL CANCER Description Preinvasive cancer is limited to the cervix. Invasive cancer is in the cervix and other pelvic structures. Metastasis usually confined to the pelvis, but distant metastasis occurs through lymphatic spread. Premalignant changes are described on a continuum from dysplasia, which is the earliest premalignancy change, to carcinoma in situ, the most advanced premalignancy change. OVARIAN CANCER Description Ovarian cancer grows rapidly, spreads fast, and is often bilateral. Metastasis occurs by direct spread to the organs in the pelvis, by distal spread through lymphatic drainage, or by peritoneal seeding. Prognosis is usually poor because the tumor usually is detected late. An exploratory laparotomy is performed to diagnose and stage the tumor. BREAST CANCER Description Breast cancer is classified as invasive when it penetrates the tissue surrounding the mammary duct and grows in an irregular pattern. Metastasis occurs via lymph nodes. Common sites of metastasis are the bone, lungs; metastasis also occurs to the brain and liver. Diagnosis is made by breast biopsy through a needle aspiration or by surgical removal of the tumor with microscopic examination of the malignant cells. Surgical Breast Procedures Lumpectomy Tumor is excised and removed. Lymph node dissection may also be performed. Simple Mastectomy Breast tissue and the nipple are removed. Lymph nodes are left intact. Modified Radical mastectomy Breast tissue, nipple, and lymph nodes are removed. Muscles are left intact. Halsted Radical Mastectomy Breast tissue, nipple, underlying muscles, and lymph nodes are removed. BRONCHOGENIC CARCINOMA is a malignant neoplasm of the lung arising from the epithelium of the bronchus or bronchiole. Pathology Bronchogenic carcinomas begin as a small focus of atypical epithelial cells within the bronchial mucosa. As the lesion progresses, the atypia becomes frankly malignant and the neoplasm grows in size. The neoplasm may grow into the bronchial lumen, along the mucosa or into the bronchial wall and adjacent lung parenchyma. Eventually the neoplasm spreads to regional lymph nodes and distant organs such as

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the liver, brain and bone. Most bronchogenic carcinomas form a mass in or near the hilus. Some neoplasms, especially the adenocarcinomas, form a mass in the periphery of the lung. Squamous Cell Carcinoma: The neoplasm is composed of malignant squamous cells which may vary in degree of differentiation from tumor to tumor. A well differentiated squamous cell carcinoma may form keratin and intercellular bridges. Adenocarcinoma: The neoplasm is composed of malignant glandular epithelium which may vary in degree of differentiation from tumor to tumor. Well differentiated neoplasms may form distinct glands, other neoplasms may vary from forming papillary structures to solid neoplasms without any gland formation. Adenocarcinomas tend to be smaller than other bronchogenic carcinomas and located in the periphery of the lung. A distinctive type of adenocarcinoma is bronchioloalveolar carcinoma. CLINICAL NOTE: This neoplasm is the most common type in women and nonsmokers. Small cell carcinoma: The neoplasm is composed of small cells containing dark blue, round nuclei and sparse cytoplasm. These cells resemble (but are not) lymphocytes and are arranged in clusters. CLINICAL NOTE: This neoplasm is strongly related to smoking. It is a very aggressive neoplasm, generally having metastasized at the time of diagnosis. Large cell carcinoma: The neoplasm is composed of large, undifferentiated malignant cells. Bronchioloalveolar carcinoma: The neoplasm is a distinctive form of adenocarcinoma. The neoplasm arises from the epithelium of the terminal bronchiole or the alveolus. The neoplastic cells are columnar, lining alveoli or form palliary growths which project into the alveolus. Pathophysiology Bronchogenic carcinoma tends to form an intraluminal mass which may partially or completely obstruct the bronchus. The neoplasm also may compress or invade local structures such as aorta, esophagus, superior vena cava or cervical sympathetic chain. Bronchogenic carcinoma may present with a variety clinical manifestations but the major findings are cough, weight loss, chest pain and dyspnea. These neoplasms also have the capacity to secrete hormones or hormone-like substances which have a variety of clinical effects. LIVER CANCER Primary liver cancer begins in the cells of the liver itself. Although many cancers are declining, new cases of primary liver cancer are increasing. Cancer affecting the liver is more commonly metastatic cancer, which occurs when tumors from other parts of the body spread (metastasize) to the liver. Cancers that commonly spread to the liver include colon, lung and breast cancers. These cancers aren't called liver cancer. Instead, they are named after the organ in which the cancer began such as metastatic colon cancer to describe cancer that begins in the colon and spreads to the liver. These metastatic cancers are treated based on where the cancer began, rather than being treated as primary liver cancers. Primary liver cancer is rarely discovered early and often doesn't respond to current treatments thus, the prognosis is often poor. Even when treatments fail to provide much improvement in the liver cancer itself, pain and other signs and symptoms caused by liver cancer can be aggressively treated to improve quality of life. But the most important news about primary liver cancer is that you can greatly reduce your risk by protecting yourself from hepatitis infection and cirrhosis, the leading causes of the disease. Liver cancer occurs when liver cells begin to grow abnormally. It's not completely understood why this happens, but researchers believe that cancer starts with damage to DNA the material that contains the instructions for every chemical process in your body, including the rate of cellular growth. DNA damage causes changes in these instructions. One result is that cells may begin to grow out of control and eventually form a tumor a mass of malignant cells. Primary liver cancer is divided into several types based on the type of cells that become cancerous. Types include: Hepatocellular carcinoma (HCC). This is the most common form of primary liver cancer in both children and adults. It starts in the hepatocytes, the main type of liver cell. Cholangiocarcinoma. This type of cancer begins in the small tube-like bile ducts within the liver. This type of cancer is sometimes called bile duct cancer. Hepatoblastoma. This rare type of liver cancer affects children younger than 4 years of age. Most children with hepatoblastoma can be successfully treated. Angiosarcoma or hemangiosarcoma. These rare cancers begin in the blood vessels of the liver and grow very quickly. Risk factors Primary liver cancer can affect people of all ages and races, but certain factors may increase your risk, including: Sex. Men are more likely to develop liver cancer than are women, though it isn't clear why. Age. In the United States and Europe, liver cancer diagnosis occurs on average at about age 60. People in Asia and Africa tend to be diagnosed with liver cancer at younger ages between 20 and 50.

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Chronic infection with HBV or HCV. Chronic infection with hepatitis B virus (HBV) or hepatitis C virus (HCV) is by far the most important risk factor for liver cancer. Cirrhosis. This progressive and irreversible condition causes scar tissue to form in your liver and increases your chances of developing liver cancer. Diabetes. People with this blood sugar disorder have a greater risk of liver cancer than do people who don't have diabetes. Having both diabetes and hepatitis C infection increases the risk even more. Exposure to aflatoxins. Consuming foods contaminated with fungi that produce aflatoxins greatly increases the risk of liver cancer. Crops such as corn, soybeans and peanuts can become contaminated with aflatoxins. Excessive alcohol consumption. Consuming more than a moderate amount of alcohol can lead to irreversible liver damage and increase your risk of liver cancer. Moderate consumption is defined as no more than two drinks a day for men and one drink for women. A drink is one 4- to 5-ounce glass of wine, 12 ounces of beer or a 1.5-ounce shot of 80-proof distilled spirits. Smoking. Smoking tobacco of any kind makes it more likely that you'll develop liver cancer. Bile duct disease. A disease called primary sclerosing cholangitis can cause inflammation and scarring of the liver's bile ducts. This increases your risk of bile duct cancer.

Screening and diagnosis 1. Screening Screening for liver cancer hasn't been definitively proved to reduce the risk of dying of liver cancer. For this reason, many medical groups don't recommend liver cancer screening. However, the American Association for the Study of Liver Diseases recommends liver cancer screening for those thought to have a high risk, including people who have: Hepatitis B and one or more of the following: Are an Asian male older than 40, Asian female older than 50 or African and older than 20, have liver cirrhosis, or have a family history of liver cancer Liver cirrhosis from alcohol use Hepatitis C An inherited form of hemochromatosis Primary sclerosing cholangitis Discuss the pros and cons of screening with your doctor. Together you can decide whether screening is right for you based on your risk. Screening typically involves blood tests and an ultrasound exam once or twice each year.

2. Diagnosis
If you experience any of the symptoms of liver cancer, your doctor will ask you about your medical history and perform a physical exam. Tests and procedures used to diagnose liver cancer include: Ultrasound (ultrasonography). This test uses sound waves to produce a picture of internal organs, including the liver. Ultrasound is painless and usually takes less than 30 minutes. While you lie on a bed or examining table, a wand-shaped device (transducer) is placed on your body. It emits sound waves that are reflected from your liver and transformed into a computer image. Ultrasound provides information about the shape, texture and makeup of tumors. Computerized tomography (CT) scan. This test uses X-rays to produce cross-sectional images of your body. You may also have a variation of the test known as a CT angiogram in which contrast dye is injected into an artery in your liver. X-rays then track the dye as it flows through the blood vessels in your liver. A CT angiogram, which may take up to an hour to perform, can provide detailed information on the number and location of liver tumors, but a CT scan exposes you to more radiation than conventional X-rays do, and some people may experience an allergic reaction to the contrast dye. Magnetic resonance imaging (MRI). MRI creates images using a magnetic field and radio waves. Sometimes a contrast dye also may be used. The test can take from 15 minutes to an hour. Newer MRIs can show images of the ducts that transport bile from the liver to the upper part of the small intestine (duodenum) as well as of the arteries and veins within the liver. Liver biopsy. In this procedure, a sample of tissue is removed from your liver and examined under a microscope. Liver biopsy is considered the only definitive way to diagnose liver cancer. Your doctor may use a thin needle or a lighted instrument (laparoscope) to obtain the sample. Biopsy carries a risk of bleeding, bruising and infection. Blood tests. Doctors sometimes use a blood test that checks for the presence of alphafetoprotein (AFP) a type of protein found in small amounts in adults to detect liver cancer. But the test isn't perfect. Not all malignant liver tumors produce AFP, and those that do may be advanced by the time protein levels become elevated. In addition, other types of cancer and even some noncancerous liver diseases can raise AFP levels.

Staging Staging tests help determine the size and location of cancer and whether it has spread. Liver cancer may be staged in different ways. One method uses the Roman numerals I through IV, with higher numbers indicating cancers that are more advanced.

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A stage I tumor is small and confined to one lobe of the liver. By stage IV, several tumors may exist in different lobes, or malignant cells may have spread to other parts of the body. Doctors may also use the following stages to describe primary liver cancer in adults: Localized resectable. At this stage, the tumor is confined to one lobe of your liver and can be completely removed in an operation. The term "resectable" refers to a tumor that can be surgically removed. Localized unresectable. The cancer is found in only one part of your liver, but can't be completely removed, either because the noncancerous portion of your liver isn't healthy or because the cancer is located near your liver's main arteries, veins and bile ducts. Advanced. This stage of cancer has spread throughout the liver or to other parts of your body, particularly the bones or lungs. You're more likely to have advanced cancer if you also have cirrhosis or chronic hepatitis. Recurrent. This means the cancer has returned to your liver or to another part of your body after being treated. Stages of primary cancer in children Doctors use the following stages to describe childhood liver cancer: Stage I. At this stage, the cancer can be removed with surgery. Stage II. Most stage II liver cancers can be removed with an operation, but microscopic amounts of cancer remain in the liver after surgery. Stage III. At this stage, some of the cancer may be surgically removed, but some will remain in the lymph nodes or abdomen. Stage IV. This stage of cancer has spread to other parts of the body. Recurrent. This means the cancer has returned after it has been treated. It may recur in the liver or in another part of the body. Complications People with liver cancer may sometimes experience the following complications: Liver failure. This occurs when the liver is no longer able to function adequately. It usually develops when there is extensive damage to liver cells. Kidney failure. The kidneys also may fail, losing their ability to filter fluids and waste and causing dangerous levels of these substances to accumulate in the body. Spread of the cancer cells (metastasis). Cancer that spreads to areas outside the liver becomes more difficult to treat. Liver cancer most commonly spreads to the lungs and bones. Treatment Treatments for primary liver cancer depend on the extent (stage) of the disease as well as your age, overall health, feelings and personal preferences. Discuss all of your options carefully with your treatment team. The goal of any treatment is to eliminate the cancer completely. When that isn't possible, the focus may be on preventing the tumor from growing or spreading. In some cases palliative care only is appropriate. Palliative care refers to treatment aimed not at removing or slowing the disease but at helping relieve symptoms and making you as comfortable as possible. Treatments for primary liver cancer in adults Treatments for adults with primary liver cancer include: Surgery. The best treatment for localized resectable cancer is usually an operation known as surgical resection. In some cases, the area of the liver where the cancer is found can be completely removed. You aren't a candidate for surgical removal of liver tumors if you have cirrhosis or only a small amount of healthy liver tissue. Even when resections are successful, there is a chance the cancer can recur elsewhere in the liver or in other areas within a few years. Alcohol injection. In this procedure, pure alcohol is injected directly into tumors, either through the skin or during an operation. Alcohol dries out the cells of the tumor and eventually the cells die. Each treatment consists of one injection, although you may need a series of injections for the best results. Alcohol injection has been shown to improve survival in people with small hepatocellular tumors. It may also be used to help reduce symptoms in cases of metastatic liver cancer. The most common side effect is leaking of alcohol onto the liver or into the abdominal cavity. Radiofrequency ablation. In this procedure, electric current in the radiofrequency range is used to destroy malignant cells. Using an ultrasound or CT scan as a guide, your surgeon inserts several thin needles into small incisions in your abdomen. When the needles reach the tumor, they're heated with an electric current, destroying the malignant cells. Radiofrequency ablation is an option for people with small, unresectable hepatocellular tumors and for some types of metastatic liver cancers. Although the procedure has a somewhat higher risk of serious complications than alcohol injection does, it appears to provide better outcomes. Chemoembolization. Chemoembolization is a type of chemotherapy treatment that supplies strong anti-cancer drugs directly to the liver. Chemoembolization isn't curative, but it can shrink tumors in a certain percentage of people, which may provide symptom relief and improve survival. During the procedure, the hepatic artery the artery from which liver cancers derive their blood supply is blocked, and chemotherapy drugs are injected between the blockage and the liver. The idea is that by targeting the tumor directly, doctors can use potent doses of drugs

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without creating as many side effects as occur with systemic chemotherapy. But the fact is that chemoembolization causes many of the same side effects as other forms of chemotherapy, including abdominal pain, nausea and vomiting. Chemoembolization is less likely to cause some side effects such as lowered blood cell counts or hair loss. Cryoablation (cryosurgery or cryotherapy). This treatment uses extreme cold to destroy cancer cells. Cryoablation may be an option for people with inoperable primary and metastatic liver cancers. It may also be used in addition to surgery, chemotherapy or other standard treatments. During the procedure, your doctor places an instrument (cryoprobe) containing liquid nitrogen directly onto liver tumors. Ultrasound images are used to guide the cryoprobe and monitor the freezing of the cells. Side effects include damage to the bile ducts and major blood vessels, leading to bleeding or infection. Radiation therapy. This treatment uses high-powered energy beams to destroy cancer cells and shrink tumors. Radiation may come from a machine outside your body or from radiationcontaining materials inserted into your liver. Radiation may be used on its own to treat localized unresectable cancer. Or you may have radiation therapy following surgical removal of a tumor to help destroy any remaining malignant cells. Radiation side effects may include fatigue, nausea and vomiting. Chemotherapy. This treatment uses powerful drugs to kill cancer cells. Chemotherapy may be systemic meaning it travels throughout your body in your bloodstream or regional. Systemic chemotherapy is generally not effective in treating liver cancer, but may be a treatment option in certain cases. Liver transplantation. In this surgical procedure, a diseased liver is removed and replaced with a healthy, donated organ. Liver transplantation may be an option for some people with small, early-stage liver tumors and for certain people with bile duct tumors. In other cases, especially when tumors are larger or blood vessels are involved, a transplant may not improve long-term outlook because the cancer may recur outside the new liver. Sorafenib (Nexavar). Sorafenib was approved by the Food and Drug Administration in 2007 for use in advanced inoperable liver cancer. Sorafenib is a targeted therapy designed to interfere with a tumor's ability to generate new blood vessels. Sorafenib has been shown to slow or stop advanced liver cancer from progressing for a few months longer than with no treatment. More studies are needed to understand how targeted therapies may be used to control advanced liver cancer.

Treatments for primary liver cancer in children Liver cancer in young people is rare. As a result, most children with the disease are treated at centers that specialize in childhood cancers. In general, the treatments available for children are the same as for adults, and the best approach depends on the stage and type of cancer as well as the child's age and overall health. Clinical trials Because standard treatments often aren't effective in treating liver cancer, you may want to consider participating in a clinical trial a research study that tries to improve current treatments or find new treatments. This can give you access to experimental therapies that might not otherwise be available. There are no guarantees with clinical trials, however, and you should fully understand the potential risks as well as possible benefits before taking this step. Prevention In many cases it's not possible to prevent the spread of cancer from another site to the liver. And it may not always be possible to prevent primary liver cancer. But you can greatly reduce your risk by taking steps to protect yourself from hepatitis B and C, cirrhosis and other liver diseases.

Get vaccinated. The single most effective way to prevent hepatitis B is to receive the hepatitis B
vaccine, which provides more than 90 percent protection for both adults and children. Protection lasts years and may even be lifelong. The vaccine can be given to almost anyone, including infants, older adults and those with compromised immune systems. Infants often receive the vaccine in the first year of life typically at 2, 4 and 9 months of age.

Take measures to prevent hepatitis C. Because no vaccine for hepatitis C exists, the following
measures can play a key role in protecting your health: Educate yourself and others. Make sure you understand what viral hepatitis is and how it's transmitted. Know the health status of any sexual partner. Don't engage in unprotected sex unless you're absolutely certain your partner isn't infected with HBV, HCV or any other sexually transmitted disease. If you don't know the health status of your partner, use a new latex condom every time you have vaginal or anal sex. If you don't have a male condom, use a female condom. Don't use IV drugs, but if you do, use a clean needle. The best way to protect yourself from HCV is not to inject drugs. But if that isn't an option for you, make sure any needle you use is sterile, and don't share it. Contaminated drug paraphernalia is responsible for about half of all new hepatitis C cases. Take advantage of needle exchange programs in your community and consider seeking help for your drug use. Avoid body piercing and tattooing. Needles that may not be properly sterilized can spread the virus.

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Be cautious about blood products in certain countries. Most Americans with HCV became infected through blood transfusions received before 1992 the year improved blood-screening tests became available. Although the blood supply is now well screened in the United States, this isn't always the case in other countries. If an emergency requires that you receive blood or blood products in another country, get tested for HCV and HBV as soon as you return home. Avoid or limit alcohol. Alcohol speeds the progression of any liver disease you may have and is the leading cause of cirrhosis a key factor in primary liver cancer. Avoid medications that may cause liver damage. Your doctor can advise you about these medications, which may include over-the-counter medications as well as prescription drugs. Avoid mixing alcohol and acetaminophen (Tylenol, others) a combination known to cause liver damage. Avoid exposure to environmental toxins. Your liver filters every substance you ingest, inhale or apply to your skin. For that reason, avoid unnecessary chemical exposure.

Coping skills Learning you have any life-threatening illness can be devastating. But coping with a diagnosis of liver cancer can be especially difficult. The more advanced the disease when it's discovered, the less likely the chance of cure. As a result, you may feel overwhelmed just when you need to make crucial decisions. You're also likely to be even more concerned about others than yourself. How will you tell your children? Will your partner be able to cope? Who will take care of all of the things you normally do if you can't? Although there are no easy answers for people dealing with liver cancer, some of the following suggestions may be of help: Learn all you can about your illness. Learn everything you can about liver cancer how the disease progresses, your prognosis and your treatment options, including both experimental and standard treatments and their side effects. Be sure you understand whether a particular approach is used to treat cancer or provide palliative care. Don't be afraid to seek a second opinion and to explore treatments available through clinical trials. You will have many decisions to make in the weeks and months ahead. The more you know the more active role you can take in the decision-making process. In addition to talking to your medical team, look for information in books and reputable sources on the Internet. The National Cancer Institute offers a toll-free information line called the Cancer Information Service. It provides access to trained counselors and accurate, up-to-date information on all aspects of living with cancer. You can reach the Cancer Information Service 24 hours a day at 800-4-CANCER, or 800-4226237. Maintain a strong support system. Strong relationships are crucial in dealing with lifethreatening illnesses. Although friends and family can be your best allies, in some cases they may have trouble dealing with your illness. Or you may not have a large social network. If so, the concern and understanding of a counselor, medical social worker or even a formal support group can be helpful. Although support groups aren't for everyone, they can sometimes be a good resource for practical information about your disease. You may also find strength and encouragement in being with people who are facing the same challenges you are. If you're interested in learning more about support groups, talk to a doctor, nurse, social worker or psychologist. They may be able to put you in touch with a group in your area. Or check your local phone book, library or a cancer organization. The National Cancer Institute also can provide a list of support groups. After deciding to participate in a group, try it out a few times. If it doesn't seem useful or comfortable, you don't have to continue. Come to terms with your illness. Coming to terms with your illness may be the hardest thing you've ever done. For some people, having a strong faith or a sense of something greater than themselves makes this process easier. Others seek counseling from someone who understands life-threatening illnesses, such as a medical social worker, psychologist or chaplain. Many people also take steps to ensure that their end-of-life wishes are known and respected.

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