Title: Pediatric Malignancies
1Pediatric Malignancies
- Jan Bazner-Chandler
- CPNP,MSN, CNS, RN
2Pediatric Malignancies
- 1 of all cancers
- Involves tissues of
- CNS, bone, muscle, endothelial tissue
- Grows in a short period of time
3Causes
- Genetic alteration
- Environmental influences
- No know prevention
- Metastasic disease seen in 80
4Response to Treatment
- Very responsive to chemotherapy
- More than 60 cure rate
5Classification of Tumors
- Embryonal tumor arises from embryonic tissue
- Lymphomas lymphatic tissue
- Leukemias blood
- Sarcoma seen in bone, cartilage, nerve and fat
6Cardinal 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.
7Diagnostic Tests
- X-ray
- Skeletal survey
- CT scan
- Ultrasound
- MRI
- Bone marrow aspiration
8Biopsy
- Identify cell to determine type of treatment
9Treatment Modalities
- Determined by
- Type of cancer
- Location
- Extent of disease
10Surgery
- 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
11Radiation 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.
12Chemotherapy
- 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.
13Chemotherapy
- 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.
14Chemotherapy
- 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.
15Administration
- 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
16Goals of Chemotherapy
- Reducing the primary tumor size
- Destroying cancer cells
- Preventing metastases and microscopic spread of
the disease
17Chemotherapy 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
18Bone 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
- Parents stem cells
19Gene Therapy
- Use of gene therapy in the treatment of childhood
cancer is promising yet complex and still in
early phases of clinical application.
20Management 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
21Pain Management
- Pain caused by disease
- Pain with procedures and treatments
- Pain associated with side effects of treatment
22Pain Management
- Pharmacologic
- Non-Pharmacologic
- Sedation or anesthetic medications
- EMLA cream
- Conscious sedation
23(No Transcript)
24Pain Control
- http//pedspain.nursing.uiowa.edu/
25Immunosuppression 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.
26Neutropenia
- 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.
27Treatment 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
28Varicella
- 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.
29Varicella Immunizations
- Three months after chemotherapy
- Off prednisone
- Many will have already had the immunization as a
toddler since it is now a required immunization.
30Central Venous Access Devices
- Two decades ago, CVAD were introduced as an
integral part of the pediatric oncology patients
treatment plan. - Used to deliver chemotherapy, blood components,
antibiotics, fluids, TPN, medications and blood
sampling.
31CVAD 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
32Chemotherapy 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.
33Management of Side Effects
34Malnutrition
- 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.
35Nutrition Interventions
- Initial nutritional assessment
- History of childs 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
36Nausea 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
37Interventions
- 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. (gt3 years) - Antihistamines benadryl
- Administer before chemotherapy
38Mucositis
- 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.
39Interventions
- 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
40Constipation
- Assess normal bowel habits
- Increase fiber and fluids in diet
- Stool softeners / colace
- Physical activity
- Avoid digital manipulation
41Diarrhea
- Assess for signs of dehydration
- Record stool patterns
- IV fluids as needed
- Low-residue or lactose-free diet
- Good hand washing
42Hair 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
43Psychosocial Support
- Support groups
- Open communication
- Daily contact with oncology team
- Trusting relationship with nurse
44Growth and Development
- Promote normal G D
- Allow decision making
- Establish daily routines
- Play therapy
- Friends
- School attendance or tutor
45Leukemia
- Most common malignancy
- 4 in 100,000
- Increase in chromosome disorders
- High survival rate
46Leukemia
- Unrestricted proliferation of immature WBCs in
the blood forming tissues of the body. - The cells look different from normal cells and do
not function properly.
47Prognosis
- 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
48Diagnosis
- Peripheral blood smear
- Bone marrow analysis
- Lumbar puncture
49Peripheral Blood Smear
Red circles or RBC large blue are WBC blue dots
are platelets
http//www.fghi.com/careers/html/body_smear.html
50Bone Marrow
Normal
Acute Lymphoid Leukemia
http//alice.ucdavis.edu/imd/420a/dib/acute/index
.htm
51Clinical Manifestations
- Pallor and fever
- Lethargy
- Anorexia
- Weight loss
- Hemorrhage / petechiae
- Hepatomegly / splenomegaly
523 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.
53Induction 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
54Consolidation Phase
- Several drugs are used in combination to prevent
remaining leukemia cells from developing
resistance. - Drugs include methotrexate and 6-mercaptopurine,
vincristine and prednisone
55Maintenance
- 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.
56CNS 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. -
57Nursing Interventions
- Assess for infection
- Monitor blood values
- I O / nutrition
- Complications of chemotherapy
- Good hand washing
- Aseptic technique for blood draws
58Leukemia 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.
59Time 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.
60Time 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.
61CNS Tumors
- 2nd most common malignancy
- 65 have 5 year survival rate
- Most common tumors
- Astrocytomas 50
- Medulloblastomas 25
- Brain stem gliomas 10
-
62Clinical 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.
63Management
- Surgery if tumor accessible
- Chemotherapy
- Radiation Reserved for patient older that
2-years of age - Survival rate based on location
64Chemotherapy
- After surgery to prevent tumor from coming back
- Shrink tumor that cannot be operated on
- Shrink tumor so it can be operated on
65Chemotherapy
- 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
66Brain Tumors
Astrocytoma Large right frontal lobe neoplasm
with small area of necrosis
67Hodgkin's Disease
- 3rd most common malignancy
- 15 to 30 years
- Three times higher in males
- Excellent cure rates
68Clinical Manifestations
- Night sweats
- Weight loss
- Malaise
- Painless, firm nodes
69Treatment
- Radiation to nodes
- Chemotherapy
- Combination therapy for six months
- Prednisone
- Stem cell transplant
70Long 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
71Neuroblastoma
- 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.
72Clinical Manifestations
- Depends on site of tumor
- Diagnosis
- CT scan
- Bone scan
- 95 secrete catecholamines in the urine.
73Treatment
- 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.
74Neuroblastoma
75Wilms 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
76Clinical Manifestations
- Firm non-tender, painless mass in abdomen
- Hematuria
- Hypertension
- Do not palpate the abdomen
77CT Scan Wilms Tumor
http//www.uhrad.com/pedsarc/peds048.htm
78Wilms Tumor
79Treatment
- Surgery
- Nephrectomy
- Prevent rupture of capsule
- Sample for pathology
- Chemotherapy and radiation are given based on the
stage of the disease.
80Osteogenic 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
81Diagnosis
82Osteosarcoma Tumor
Femur has a large mass involving the metaphysis
of bone. Tumor has destroyed the cortex.
Andersons Pathology
83Treatment
- Limb salvage
- Amputation
- Chemotherapy
84Limb Salvage
http//www.clevelandclinic.org/ortho/tumor/limbSal
vageLg.htm
85Ewing Sarcoma
- Tumor of flat bones
- Pelvis, chest, vertebrae
- Rare in children under 5 years
- 75 diagnosed by age 20
86Ewing Sarcoma
87Ewing Sarcoma Tumor
Ewing Sarcoma at distal end of the tibia. tumor
extends into the soft tissue.
Andersons Pathology
88Rhabdomyosarcoma
- Most common soft bone tissue tumor
- Head and neck 40
- GU 20
- Extremities 20
- Trunk 15
89Rhabdomyosarcoma
http//www.brisbio.ac.uk/ROADS/subject-listing/fac
e.html
90Treatment
- Surgical removal
- Chemo based on tissue biopsy
- Radiation
91Retinoblastoma
- Intraocular / Embryonic tumor
- 1 in 16,000
- family history
- High incidence of malignancies
92Retinoblastoma
Pupil reflex Cat Eyes
http//www.djo.harvard.edu/meei/PI/RB/RB.html
93Retinoblastoma
http//homepage.idx.com.au/muznsam/
94Retinoblastoma
95Treatment
- Surgical enucleation of eye
- Genetic counseling
- Follow-up care up to 18 Years