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.
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.
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 Control
http://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
Bone Marrow
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.
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
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
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
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
Treatment
Limb salvage
Amputation
Chemotherapy
Limb Salvage
Ewing Sarcoma
Tumor of flat bones
Pelvis, chest, vertebrae
Rare in children under 5 years
75% diagnosed by age 20
Ewing Sarcoma Tumor
Rhabdomyosarcoma
Most common soft bone tissue tumor
Head and neck 40%
GU 20%
Extremities 20%
Trunk 15%
Rhabdomyosarcoma
Treatment
Surgical removal
Chemo based on tissue biopsy
Radiation
Retinoblastoma
Intraocular / Embryonic tumor
1 in 16,000
+ family history
High incidence of malignancies
Retinoblastoma
Treatment
Surgical enucleation of eye
Genetic counseling
Follow-up care up to 18 Years
