Pediatric Malignancies
Jan Bazner-Chandler
CPNP,MSN, CNS, RN
Pediatric Malignancies 1% of all cancers
Involves tissues of:
CNS, bone, muscle, endothelial tissue
Grows in a short period of time
Causes Genetic alteration
Environmental influences
No know prevention
Metastasic disease seen in 80%
Response to Treatment Very responsive to chemotherapy
More than 60% cure rate
Classification of Tumors Embryonal tumor arises from embryonic tissue
Lymphomas = lymphatic tissue
Leukemias = blood
Sarcoma = seen in bone, cartilage, nerve and fat
Cardinal Signs of Cancer Unusual mass or swelling Unexplained paleness and loss of energy Spontaneous bruising Prolonged, unexplained fever Headaches in morning Sudden eye or vision changes Excessive – rapid weight loss.
Diagnostic Tests X-ray Skeletal survey CT scan Ultrasound MRI Bone marrow aspiration
Biopsy Identify cell to determine type of treatment
Treatment Modalities Determined by:
Type of cancer
Location
Extent of disease
Surgery The oldest form of cancer treatment Surgery plays important role in initial
diagnosis: biopsy of primary tumor. Excision of tumor when possible Facilitating treatment: insertion of catheters
for long-term treatment
Radiation Therapy The use of ionizing radiation to break apart bonds
within a cell causing cell damage and death. External beam therapy accounts for the majority of
radiation treatments in children. Problems: radiation beams cannot distinguish
between malignant cells and healthy cells.
Chemotherapy Primary treatment modality used to cure
many pediatric cancers. Chemotherapy is the use of drugs to destroy
cancer cells. The destruction is accomplished by inhibiting
cells within the body to divide, which eventually leads to cell death.
Chemotherapy Can be given in addition to another form of
therapy such as radiation or surgery. Drugs may be administered before surgery to
reduce size of tumor. Adjuvant chemotherapy is used after surgery
or radiation therapy to prevent relapse.
Chemotherapy Combination chemotherapy is the use of
more than one class of drug. Administering different classes of chemo
drugs ensures a greater chance of achieving complete cancer cell destruction and achieving remission.
Administration Chemotherapy can be given by mouth,
subcutaneous or intramuscular injections, intravenously, or intrathecally. Oral route used if drug is well absorbed and non
irritating to the GI tract Sub-q or IM: Slow systemic release IV push, piggyback or intravenous infusion
Goals of Chemotherapy Reducing the primary tumor size Destroying cancer cells Preventing metastases and microscopic
spread of the disease
Chemotherapy Drugs Alkylating drug: attack DNA Antimetabolites: interfere with DNA production Antitumor antibiotics: interferes with DNA production Plant alkaloids: prevent cells from dividing Steroid hormones: slow growth of some cancers
Bone Marrow Transplant HSCT: Hematopoictic Stem Cell Transplant:
CHLA has one of the largest program. The option of HSCT depends on the patients
disease, disease status, and general physical condition.
Involves: Umbilical cord blood Parent’s stem cells
Gene Therapy Use of gene therapy in the treatment of
childhood cancer is promising yet complex and still in early phases of clinical application.
Management of Cancer Patient / family education
Begins at time of diagnosis Continues through treatment phases Maintained in post-survival years Support if death of child
Emotional aspects of leukemia http://leukemia.org/pages/413.html
Pain Management Pain caused by disease
Pain with procedures and treatments
Pain associated with side effects of treatment
Pain Management Pharmacologic
Non-Pharmacologic
Sedation or anesthetic medications EMLA cream Conscious sedation
Pain Controlhttp://pedspain.nursing.uiowa.edu/
Immunosuppression and Infection Children with cancer become immune impaired
from a number of causes: Lymphocyte production is altered Splenic dysfunction can prevent maturation of blood
cells and alteration is inflammatory response. Cancer therapy can decrease immunoglobulin
concentrations.
Neutropenia Significant neutropenia can develop during
chemotherapy creating an increased risk of infection in the child with cancer.
Neutropenia occurs when the absolute neutrophil count decreases below 500.
Treatment of Neutropenia Granulocyte colony stimulating factor
decreases the duration of neutropenia by stimulating the proliferation of the progenitor cells of the granulocytes, specifically the neutophils.
G-CSF: 5mcg/kg/day given subcutaneous
Varicella If an immunosuppressed child with no history
of varicella infection or varicella immunization has direct contact with an individual with chickenpox or shingles, varicella zoster immune globulin should be administered.
Acyclovir IV is used in some cases.
Varicella Immunizations Three months after chemotherapy Off prednisone Many will have already had the immunization
as a toddler since it is now a required immunization.
Central Venous Access Devices Two decades ago, CVAD were introduced as
an integral part of the pediatric oncology patient’s treatment plan.
Used to deliver chemotherapy, blood components, antibiotics, fluids, TPN, medications and blood sampling.
CVAD Infection Prevention• Teach family to report signs of catheter
infections: fever, chills, swelling, pain, drainage, or erythema.
• Aseptic technique for dressing changes and heparin flushing.
• Avoid trauma to device• Observe for catheter occlusion
Chemotherapy Side Effect Drugs affect not only the cancer cells but also
healthy cells. Cells most affected are rapidly growing cells
such as hair follicles, reproductive system, bone marrow and gastrointestinal tract.
Management of Side Effects
Malnutrition Occurs in 8 to 32% of the pediatric oncology
population Nutritional goals focus on maintaining normal
growth and development as well as preventing nutritional deficiencies.
Nutrition Interventions Initial nutritional assessment History of child’s eating habits, food allergies,
use of nutritional supplements, base line weight and height measurements.
Enteral feedings at night: preserve intestinal mucosa by keeping it functional
Nausea and Vomiting Most common side effect of cancer treatment. Chemotherapy-associated vomiting is a reflex
controlled by chemoreceptor trigger zone that stimulates the vomiting center in the brain.
Tumor location Radiation therapy Anticipatory nausea
Interventions Antiemetics such as Phenothiazines: (Trilafon),
(Phenergan)and (Thorazine) block dopamine receptors from stimulating the chemoreceptor trigger zones.
Serotonin-receptor antagonist such as Granisetron (Kytril) and Ondansetron (Zofran) are very effective. (>3 years)
Antihistamines: benadryl Administer before chemotherapy
Mucositis Progressive, inflammatory, ulcerative condition of the oral
and gastric mucosa. Occurs due to the interruption of cell renewal process of
the epithelium leading the mucosal atrophy and ulceration Thrombocytopenia or physical trauma may lead to bleeding
and further mucosal damage. Neutropenia and poor dental hygiene predisposes the oral
mucosa to secondary infection.
Interventions Baseline assessment including the oral
cavity, teeth, and gingival mucosa. History of dental exam and use of orthodontic
appliances Meticulous oral care Mouth rinses Monitor hydration status
Constipation Assess normal bowel habits Increase fiber and fluids in diet Stool softeners / colace Physical activity Avoid digital manipulation
Diarrhea Assess for signs of dehydration Record stool patterns IV fluids as needed Low-residue or lactose-free diet Good hand washing
Hair Loss More important in the older child. Most patients will experience hair loss within
10 days of induction chemotherapy Prepare patient for hair loss Males: shave hair Females: short hair style – pick out wig
Psychosocial Support Support groups
Open communication
Daily contact with oncology team
Trusting relationship with nurse
Growth and Development Promote normal G & D Allow decision making Establish daily routines Play therapy Friends School attendance or tutor
Leukemia Most common malignancy
4 in 100,000
Increase in chromosome disorders
High survival rate
Leukemia Unrestricted proliferation of immature WBC’s
in the blood forming tissues of the body.
The cells look different from normal cells and do not function properly.
Prognosis Initial WBC most significant The higher the count the poorer the
outcomes Greater than 100,000 WBC count = poor outcome
Children under 2 years and older than 10 Girls do better than boys
Diagnosis Peripheral blood smear
Bone marrow analysis
Lumbar puncture
Peripheral Blood Smear
Red circles or RBC; large blue are WBC; blue dots are platelets
http://www.fghi.com/careers/html/body_smear.html
Bone Marrow
http://alice.ucdavis.edu/imd/420a/dib/acute/index.htm
Normal Acute Lymphoid Leukemia
Clinical Manifestations Pallor and fever Lethargy Anorexia Weight loss Hemorrhage / petechiae Hepatomegly / splenomegaly
3 Phase Treatment Induction: goal is to achieve remission last
about a month Consolidation: most intensive phase of
chemotherapy lasts 4 to 8 months Maintenance: last two to three years If leukemia cells are detected in bone marrow
process is started all over again.
Induction Therapy Goal of therapy is to achieve remission Leukemia cells are no longer found in the bone
marrow samples, the normal cells return and blood counts become normal.
Drugs used: L-asparaginase, vincristine and a steroid (dexamethasone), for high-risk children a fourth drug (daunorbucin) is often used
Consolidation Phase Several drugs are used in combination to
prevent remaining leukemia cells from developing resistance.
Drugs include: methotrexate and 6-mercaptopurine, vincristine and prednisone
Maintenance If leukemia continues to be in remission
maintenance therapy can be started. Two drugs: vincristine and steroids over a
brief period every 4 to 8 weeks. Duration of total therapy 2 to 3 years.
CNS Therapy CNS prophylaxis is initiated at diagnosis and is used
to reduce the risk for CNS disease. Preventive CNS is based on the premise that the
CNS provides a sanctuary site for leukemic cells that are undetected at diagnosis and reside protected from the action of systemic therapy by the brain blood barrier.
Nursing Interventions Assess for infection Monitor blood values I & O / nutrition Complications of chemotherapy Good hand washing Aseptic technique for blood draws
Leukemia Time Line 1962 cure rate for pediatric cancer is 4 %. 1971 – A combination of chemotherapy and cranial
irradiation proves it can cure at least half of all children with ALL.
1975 – A new combination of chemo drugs helps patients with reccurrence of the disease.
Time Line
1991 Long-term survival rate increases to 73% with intensive induction therapy followed by two years of treatment with eight anti cancer drugs used on a rotating basis.
Time Line 1997 – Bone marrow transplants from
unrelated, genetically matched donors are effective against many childhood leukemia's.
1998 – Study reveals the cure rate for All has increased from 73% to 80%.
CNS Tumors• 2nd most common malignancy• 65% have 5 year survival rate• Most common tumors:
• Astrocytomas 50%• Medulloblastomas 25%• Brain stem gliomas 10%
Clinical Manifestations Classic signs and symptoms are indicative of
increased intracranial pressure. Pressure is due to tumor mass compressing vital
structure, blockage of cerebrospinal fluid flow or tumor associated edema. Gait changes / ataxia Headache with or without vomiting Blurred vision, or diplopia Forceful vomiting upon rising in the morning or
papilledema.
Management Surgery if tumor accessible Chemotherapy Radiation = Reserved for patient older that 2-
years of age Survival rate based on location
Chemotherapy After surgery to prevent tumor from coming
back Shrink tumor that cannot be operated on Shrink tumor so it can be operated on
Chemotherapy Blood brain barrier – natural filter within the body
that allows certain substances through from the blood to the brain tissues.
Drugs used are: temozolamide, procarbazine or lomustine
Methotrexate is injected intra-thecal Implantable wafers: drug is fixed with gel wafer –
drug is slowly released into brain over 2 to 3 weeks
Brain Tumors
Astrocytoma
Large right frontal lobe
neoplasm with small
area of necrosis
Hodgkin's Disease
3rd most common malignancy
15 to 30 years
Three times higher in males
Excellent cure rates
Clinical Manifestations Night sweats
Weight loss
Malaise
Painless, firm nodes
Treatment Radiation to nodes Chemotherapy
Combination therapy for six months Prednisone
Stem cell transplant
Long Term Side Effects Infertility: drugs can damage ovaries or testicles Second cancers: small risk for leukemia in future Heart disease: some drugs can cause heart
problems or radiation to middle of chest Lung damage: pneumonitis from bleomycin
Neuroblastoma Approximately 600 new cases a year. Embryonic tumor Average age of diagnosis is 2 years. Poorest survival rate 50 to 60% have metastases at time of
diagnosis.
Clinical Manifestations Depends on site of tumor
Diagnosis CT scan Bone scan 95% secrete catecholamines in the urine.
Treatment Determined by the stage of disease and age
of child. Children who have localized disease and
complete response to treatment are more likely to achieve a disease free state and long-term survival.
Neuroblastoma
Wilm’s Tumor Most common type of renal tumor in children Approximately 460 new cases each year. Children with hypospadius or cryptorchidism have a
slightly higher incidence. African American and Females at highest risk
Clinical Manifestations Firm non-tender, painless mass in abdomen
Hematuria
Hypertension
Do not palpate the abdomen
CT Scan Wilm’s Tumor
http://www.uhrad.com/pedsarc/peds048.htm
Wilm’s Tumor
Treatment Surgery
Nephrectomy Prevent rupture of capsule Sample for pathology
Chemotherapy and radiation are given based on the stage of the disease.
Osteogenic Sarcoma Malignant tumor of bone
400 new cases each year
Peak incidence is in the second decade of life, when adolescents are gaining vertical height rapidly.
Approximately 20% have metastases at diagnosis
High rate of metastasis to lungs
Diagnosis
Osteosarcoma Tumor
Femur has a largemass involvingthe metaphysis of bone. Tumor has destroyed the cortex.
Anderson’s Pathology
Treatment Limb salvage
Amputation
Chemotherapy
Limb Salvage
http://www.clevelandclinic.org/ortho/tumor/limbSalvageLg.htm
Ewing Sarcoma Tumor of flat bones
Pelvis, chest, vertebrae
Rare in children under 5 years
75% diagnosed by age 20
Ewing Sarcoma
Ewing Sarcoma Tumor
Ewing Sarcoma atdistal end of the tibia.tumor extends intothe soft tissue.
Anderson’s Pathology
Rhabdomyosarcoma Most common soft bone tissue tumor
Head and neck 40% GU 20% Extremities 20% Trunk 15%
Rhabdomyosarcoma
http://www.brisbio.ac.uk/ROADS/subject-listing/face.html
Treatment Surgical removal
Chemo based on tissue biopsy
Radiation
Retinoblastoma Intraocular / Embryonic tumor
1 in 16,000
+ family history
High incidence of malignancies
Retinoblastoma
http://www.djo.harvard.edu/meei/PI/RB/RB.html
Pupil reflex
“Cat Eyes”
Retinoblastoma
http://homepage.idx.com.au/muznsam/
Retinoblastoma
Treatment Surgical enucleation of eye
Genetic counseling
Follow-up care up to 18 Years