PAEDIATRIC MALIGNANCIES

MANALI H SOLANKI

S.Y.M.Sc.NURSING

J G COLLEGE OF NURSING

INTRODUCTION :• Leukemia is the most common

type of cancer in children. All cancers begin in cells of the body, and leukemia is a cancer that begins in blood cells. Normally, cells grow and divide to form new cells as the body needs them.

• When cells grow old, they die and new cells take their place. Sometimes, this process does not work right. In cancer, new cells form when the body does not need them, and old cells do not die when they should.

ANATOMY AND PHYSIOLOGY

DEFINITION• Leukemia (Greek leukos "white";

aima "blood") is a cancer of the blood or bone marrow characterized by an abnormal increase of blood cells, usually leukocytes (white blood cells).

CAUSES• Exposure to large amounts of radiation• Exposure to certain chemicals at work,

such as benzene. • Some types of chemotherapy to treat

another cancer• Have Down syndrome or some other

genetic problems• Smoking• Hereditary factors such as the twins

one egg

PATHO PHYSIOLOGY

Unknown aetiology

Somatic mutation in gene

Deactivate tumour suppressor gene

Malignant transformation of lymphoblast in the bone marrow

Uncontrolled proliferation of lymphoblast in bone marrow

Lymphoblasts replace the normal marrow element

Leukemia

SIGN AND SYMPTOMS

• Fever and night sweats. • Headaches• Bruising or bleeding easily. • Bone or joint pain

• A swollen or painful belly from an enlarged spleen

• Swollen lymph nodes in the armpit, neck, or groin.

• Getting a lot of infections. • Feeling very tired or weak. • Losing weight and not feeling

hungry.

TYPES OF LEUKEMIA

• Leukemia can be divided in to 4 types. Leukemia is either chronic (which usually gets worse slowly) or acute (which usually gets worse quickly):

 • Chronic Leukemias

In chronic leukemia, the leukemia cells come from mature, abnormal cells. The cells thrive for too long and accumulate.The cells grow slowly.

Acute Leukemias

Acute leukemia,on the other hand, develop from early cells, called "blasts". Blasts are young cells, that divide frequently. In acute leukemia cells, they don't stop dividing like their normal counterparts do.

The remain two types refer to the type of cells in which the leukemia started from.

Myelogenous Leukemia

• Myelogenous leukemia develops from myeloid cells. The disease can either be chronic or acute, referred as chronic myelogenous leukemia(CML), or acute myelogenous leukemia(ALL).

Lymphocytic Leukemia

• Lymphocytic leukemia develops from cells called lymphoblasts or lymphocytes in the blood marrow. The disease can be acute or chronic, referred as chronic lymphocytic leukemia (CLL), or acute lymphocytic leukemia (ALL).

DIAGNOSTIC TESTS• Physical exam: Checks for swollen

lymph nodes, spleen, or liver.• Blood tests: The complete blood count

to check the number of white blood cells, red blood cells, and platelets. Leukemia causes a very high level of white blood cells. It may also cause low levels of platelets and hemoglobin, which is found inside red blood cells.

Bone marrow aspiration or Biopsy

Cytogenetics

Spinal tap

TREATMENT

• Chemotherapy: Chemotherapy is the major form of treatment for leukemia. This drug treatment uses chemicals to kill leukemia cells. Depending on the type of leukemia client may receive a single drug or a combination of drugs. These drugs may come in a pill form, or they may be injected directly into a vein.

• Biological therapy Biological therapy works by

helping immune system recognize and attack leukemia cells.

• Targeted therapy:

Targeted therapy uses drugs that attack specific vulnerabilities within cancer cells. For example, the drug imatinib (Gleevec) stops the action of a protein within the leukemia cells of people with chronic myelogenous leukemia. This can help control the disease.

Radiation therapy

• Radiation therapy uses X-rays or other high-energy beams to damage leukemia cells and stop their growth. client may receive radiation in one specific area of body where there is a collection of leukemia cells, or client may receive radiation over whole body. Radiation therapy may be used to prepare for a stem cell transplant.

Stem cell transplant

• A stem cell transplant is a procedure to replace diseased bone marrow with healthy bone marrow. Before a stem cell transplant, client receive high doses of chemotherapy or radiation therapy to destroy diseased bone marrow. Then client receive an infusion of blood-forming stem cells that help to rebuild bone marrow.

NURSING MANAGEMENT

• Risk for Infection related to neutropenia or leukocytosis secondary to leukemia or treatment

• Risk for Haemorrhage related to thrombocytopenia secondary to either leukaemia or treatment

• Fatigue related to side effects of treatments, low haemoglobin levels, pain, lack of sleep, or other causes

• Imbalance Nutrition: Less Tan Body Requirements related to diseased condition evidenced by anorexia, pain or fatigue.

• Risk for Ineffective Family Therapeutic Regimen Management related to increased fear of death, recurrent hospitalisations etc.

LYMPHOMA

DEFINITION

• Lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system.

Generally, lymphoma is divided into two categories:• Hodgkin's disease• non-Hodgkin's lymphoma

CAUSES

• The specific cause of lymphoma is unknown. It is possible that a genetic predisposition and exposure to viral infections may increase the risk for developing Hodgkin lymphoma. There is a slightly increased chance for Hodgkin lymphoma to occur in siblings of patients.

• There has been much investigation into the association of the Epstein-Barr virus (EBV), which causes the infection mononucleosis. This virus has been correlated with a greater incidence of children diagnosed with Hodgkin lymphoma, although the direct link is unknown.

• Exposure to certain pesticides, herbicides, and solvents such as benzene has been associated with lymphoma.

SIGN AND SYMPTOMS

• painless swelling of the lymph nodes in neck, underarm, groin, and/or chest

• difficulty breathing (dyspnea) due to enlarged nodes in the chest

• fever• night sweats• tiring easily (fatigue)• weight loss/decreased appetite• itching skin (pruritus)• frequent viral infections (i.e.,

cold, flu, sinus infection)

DIAGNOSTIC TESTS

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

Biopsy

• The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer

• Excisional biopsy: The removal of an entire lymph node or lump of tissue.

• Incisional biopsy: The removal of part of a lump, lymph node, or sample of tissue.

• Core biopsy: The removal of tissue or part of a lymph node using a wide needle.

• Fine-needle aspiration (FNA) biopsy: The removal of tissue or part of a lymph node using a thin needle.

Bone marrow aspiration and biopsy

Thoracentesis

Chest x-ray

CT scan (CAT scan)

STAGES OF LYMPHOMA

The four stages of lymphoma are:• Stage I: A single tumor which hasn't

spread.• Stage II: More than one tumor, but the

tumors are all found in lymph nodes on the same side of the diaphragm (all above or all below). Stage II can also mean that a tumor has spread to another organ, but that it is close to the original lymph node tumor.

• Stage III: More than one tumor with the tumors found on different sides (above and below) of the diaphragm. There may be tumors in the spleen or more than one tumor in nearby organs.

• Stage IV: Many tumors spread throughout an organ such as the liver or stomach, as well as in the lymph nodes.

TREATMENT

• Chemotherapy is the most common treatment for lymphomas. Depending on the type and stage of disease, various combinations of drugs are administered in cycles to reduce or eliminate cancer cells.

• Radiation therapy is sometimes used in conjunction with chemotherapy to treat large areas, or to minimize discomfort caused by cancerous lymph nodes that are affecting nearby organs or structures.

Targeted therapy

• Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow.

• Tyrosine kinase inhibitors (TKIs) block signals that tumors need to grow. Some TKIs also keep tumors from growing by preventing the growth of new blood vessels to the tumors.

Surgical and Other Procedures

• Bone marrow transplantation and peripheral blood stem cell transplantation are sometimes performed. Radioimmunotherapy, which is treatment with a radioactive substance that is linked to an antibody that will attach to the tumor when injected into the body, is being tested in clinical trials. Surgical removal of the tumor may also be performed.

NURSING MANAGEMENT

• Risk for impaired Gas Exchange• Risk for infection related to

potential bone marrow depression due to chemotherapy

• Fatigue related to side effects of treatments, low haemoglobin levels, pain, lack of sleep, or other causes

DIETARY MANAGEMENT

• Try to eliminate potential food allergens, including dairy (milk, cheese, butter), wheat (gluten), corn, soy, preservatives, and food additives.

• Eat antioxidant foods, including fruits (such as blueberries, cherries, and tomatoes) and vegetables.

• Avoid refined foods, such as white breads, pastas, and sugar.

• Eat fewer red meats and more lean meats, cold water fish, tofu (soy, if no allergy), or beans for protein.

• Use healthy oils in cooking, such as olive oil or vegetable oil.

• Reduce or eliminate trans fatty acids, found in such commercially baked goods as cookies, crackers, cakes, French fries, onion rings and processed foods.

• Avoid coffee and other stimulants, alcohol, and tobacco.

• Drink 6 - 8 glasses of filtered water daily.

RETINOBLASTOMA

ANATOMY AND PHYSIOLOGY:

DEFINITION

• Retinoblastoma is a malignancy of the retinal cell layer of the eye. The tumor is composed mainly of undifferentiated anaplastic cells -- blasts -- that arise from the nuclear layers of the retina. It is the most common eye tumor in children and it usually occurs before the age of five. It can occur in one eye (unilateral) or in both eyes (bilateral).

CAUSES

• Random Somatic Mutation• Family History

 SYMPTOMS

• Leukocoria / Cat’s eye reflex: Leukocoria results when the pupil reflects a white color rather than the normal black or red color that is seen on a flash photograph.

• A red and irritated eye: The diseased eye of a child with retinoblastoma also may become red and painful. As the tumor grows, new blood vessels may form on the front surface of the iris.

• Strabismus: A deviation of the eyes. It means the eyes are not straight or properly aligned. This results from the failure of the eye muscles to work together

• Nystagmus: Causes the eyes to look involuntarily from side to side in a rapid, swinging motion rather than staying fixed on an object or person.

• Heterochromia: Refers to a difference in coloration, usually of the iris. Is a result of the relative excess or lack of melanin (a pigment).

• Double vision

PATHO PHYSIOLOGY

DIAGNOSIS:

• Well Baby Screening • Red reflex: checking for a normal reddish-

orange reflection from the eye's retina with an ophthalmoscope or retinoscope from approximately 30 cm / 1 foot, usually done in a dimly lit or dark room.

• Corneal light reflex/Hirchberg test: checking for symmetrical reflection of beam of light in the same spot on each eye when a light is shined into each cornea, to help determine whether the eyes are crossed.

• Eye examination: checking for any structural abnormalities

• Bone marrow examination or lumbar puncture may also be done to determine any metastases to bones or the brain.

• Ultrasound (Echography)• Genetic testing

TREATMENTPhotocoagulation (Laser Therapy)

Laser therapy is performed on an outpatient basis during the EUA. Laser therapy focuses a powerful beam of light through the dilated pupil or through the wall of the eye onto the cancer and the surrounding tissue. This focused and high-powered light destroys cancerous tumors inside of the eye. The small burns produced by the laser destroy the blood supply to the tumor and cause the tumor to shrink.

Cryotherapy (Freezing Treatment)

• Cryotherapy, which freezes small tumors, is performed in conjunction with an EUA. A probe (which looks like a pen) is placed on the sclera nearest to the tumor Using a very cold gas; the tumor is frozen and thawed several times. This destroys the tumor cells and leaves a flat, pigmented scar with no signs of tumor. If tumor is evident, the treatment will need to be repeated to successfully destroy all of the tumor cells.

• Transpupillary thermotherapy: Laser energy (through the use of infrared light) heats up cancer cells and surrounding blood vessels, which kills the cells.

• Brachytherapy: Radioactive material (little rods or pellets) is placed within the tumor to deliver beams of radiation to specific areas. This form of treatment minimizes the damage to surrounding healthy tissue.

Chemotherapy

Vincristine (Vincasar, Oncovin PFS)

Cycle-specific and phase-specific, which blocks mitosis in metaphase. Binds to microtubular protein, tubulin, GTP dependent. Blocks ability of tubulin to polymerize to form microtubules, which leads to rapid cytotoxic effects and cell destruction.

• Carboplatin (Paraplatin)

Inhibits both DNA and RNA synthesis. Binds to protein and other compounds containing SH group. Cytotoxicity can occur at any stage of the cell cycle, but cell is most vulnerable to action of these drugs in G1 and S phase.

• Etoposide (Toposar, VePesid)

Blocks cells in the late S-G2 phase of the cell cycle

• Immunosuppressants

The addition of cyclosporine in combination with chemotherapy regimen of carboplatin, etoposide, and vincristine reportedly have showed enhanced efficacy of chemotherapy.

• Cyclosporine (Sandimmune, Neoral)

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

Localized Plaque Radiotherapy

• In this type of treatment, a small radioactive plaque (disc) is custom made and attached to the eye. Plaque radiotherapy has advantages over external beam radiotherapy in that it delivers radiation in a more localized fashion, thereby minimizing exposure to other eye structures. It should be stressed that only a small percentage of patients with retinoblastoma are candidates for plaque radiotherapy.

Enucleation

Enucleation is the surgical removal of the eyeball, leaving eye muscles and the contents of the eye socket intact. This procedure is done when there is no other way to remove the cancer completely from the eye. Unfortunately, loss of vision for the eye removed is permanent because an eye cannot be transplanted.

PREVENTION

• Screening• Genetic counseling• Monitoring for recurrence• Monitoring for other cancers

COMPLICATIONS

• Cataract formation

• Secondary malignancy

• Optic nerve atrophy

• Temporal bone hypoplasia after external beam radiation

DIET PLAN• Energy (calories) should be adequate to

support growth and development and to reach or maintain desirable body weight.

• Keep total fat intake between between 25 to 35 percent of calories for children and adolescents 4 to 18 years of age, with most fats coming from sources of polyunsaturated and monounsaturated fatty acids, such as fish, nuts and vegetable oils.

• Choose a variety of foods to get enough carbohydrates, protein and other nutrients.

• Eat only enough calories to maintain a healthy weight for height and build. Be physically active for at least 60 minutes a day.

• Serve whole-grain/high-fiber breads and cereals rather than refined grain products.

• Introduce and regularly serve fish as an entrée. Avoid commercially fried fish.

• Serve fat-free and low-fat dairy foods.

NURSING MANAGEMENT• Ineffective peripheral tissue perfusion

related to low haemoglobin count secondary to anemia

• Risk for Infection related to operative procedure

• Acute pain related to release of prostaglandin