Transcript cancer in children - Florida Gulf Coast University

CANCER IN CHILDREN
KAREN E. MILES
FLORIDA GULF COAST UNIVERSITY
DEPARTMENT OF NURSING
CHILDHOOD CANCERS BY AGE
SITE
0-5 YR
5-10 YR
10-15 YR
Leukemia
Lymphoma
CNS
Wilms Tumor
Neuroblastoma
Retinoblastoma
Bone
Other
39.6*
03.7
15.0
09.2*
13.0*
06.4*
00.6
08.8
35.7
13.3
27.1*
06.1
02.2
01.3
05.0
05.8
22.1
16.4*
18.9
02.0
00.5
00.0
10.5*
23.6
*Peak Incidence
INCIDENCE
• 14 cases per 100,000 children/yr
• 10-25% more common in white than black
children
ETIOLOGY
• Largely unknown
• Most likely - complex interactions of both
genetic and environmental factors Ecogentics
HOST FACTORS
• Ataxiatelangectasia - autosomal recessive
transmission
• Xeroderma Pigmentosum
• Immunodeficiency States - 100-fold
increased risk, either congenital or
acquired
• Specific Congenital Anomalies - Down
Syndrome (10-18 times greater risk of
developing Leukemia)
HOST FACTORS CONT.
• Single Gene Defects - 150 associated with
development of Cancer (Fanconi Anemia &
Bloom Syndrome)
• Familial Tendencies - Sibling with Leukemia
(4 times greater chance), Monozygous
Twins (almost 100% chance)
ENVIRONMENTAL FACTORS
• Chemical and Physical Agents - 1) DES
2) Choramphenical, 3) Benzene, 4) Asbestos
• Radiation Exposure
• Anabolic Androgenic Steriods
• Cytotoxic Agents
• Immunosuppressive Agents
• Viruses - Epstein Barr
PROGNOSIS
• 70% will be cured
• Children are more responsive to tx and
better able to tolerate immediate side
effects of surgery
LEUKEMIA
• Definition - “White Blood”, Involves blood
forming tissues of the bone marrow,
spleen, and lymph nodes
• Outstanding Characteristic - Abnormal
uncontrolled proliferation of one type of
wbc
• 80-85% of childhood Leukemias are Acute
Lymphocytic Leukemia (ALL)
INCIDENCE
• 4 per 100,000 children per year
• Peak Incidence - Between 2-6 years
• Twice as common in white children as nonwhite
• More common in males
CLASSIFICATION
• Acute or Chronic
• Cell Line - Lymphoid or Myeloid Cells
• FAB Cooperative Group Classification Based on histologic appearance of
abnormal lymphoblast - 85% - L1, 15% - L2,
less than 1% - L3
CLASSIFICATION CONT.
Immunologic Classification - based on
certain surface cell antigens
• T- Cell - common in older boys
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B- Cell - Poor Prognosis
Pre B- Cell
Early Pre B- Cell (Null Cell)
Common Acute Lymphoblastic Leukemia
Antigen (CALLA)
BEST PROGNOSIS
• ALL 1
• Early Pre B- Cell
• CALLA Positive
CLINICAL MANIFESTATIONS
• Onset - Abrupt or Insidious
• Common Symptoms Reflect Bone Marrow
Failure - Decreased rbcs, decreased
platelets, and changes in wbcs
• Pallor, fatigue, petechiae, purpura, bleeding
and fever
• WBC Count of less than 10,000/mm3
CLINICAL MANIF. CONT.
• Renal Failure because of high uric acid levels
Give Allopurinal
• Extramedullary invasion of Leukemic cells
CNS most common site
MOST have at diagnosis
CLINICAL MANIF. CONT.
• Infiltration into bones and joints
• Infiltration of other organ sites: kidneys,
heart, eyes, skill and GI tract
EVALUATION
• Peripheral smear
• Blast Cell hallmark of acute leukemia
• Normal - less than 5% blast cells in bone
marrow and none in peripheral blood
• With ALL the bone marrow may be replaced
by 80-100% blast cells
TREATMENT
FOUR PHASE TREATMENT PROGRAM
• Induction Phase - Goal is no clinical
evidence of disease and normal bone
marrow. 95% in 4-6 weeks
• Intensification (Sanctuary) Therapy
• Prophylactic Therapy for the CNS
• Maintenance Therapy - Usually 3 years
PROGNOSIS
• ALL IS CURABLE
• Overall - 60-70%
• ALL1, Pre- B Cell, CALLA Positive - 90%
LYMPHOMAS
Non - Hodgkin Lymphoma
• Two Types - Nodular (Adults) and Diffuse
(Child)
• Affects 7 to 8 children per 1 million
• Rapidly progressive with enlarging
lymphoid tissue and painless
lymphadenopathy
LYMPHOMAS CONT.
• Most frequent site - Abdomen
• Treatment - Biopsy, combination
chemotherapy and radiation
• 60-80% can be cured
HODGKIN DISEASE
• Affects about 5 in 1 million children
• Mostly Adolescents
• Characterized by painless enlargement of
lymph nodes
• Presence of Sternberg-Reed cell
• Treatment Modalities - Irradiation and
chemotherapy
• 90% 10 year survival rate with localized
disease
BRAIN TUMORS
• Incidence - 2.4 per 100,000
• Cause Unknown
• CLASSIFICATION - Most arise from glial
tissue, the supportive tissue of the brain
• 60% are INFRATENTORIAL - Occur in the
posterior third of the brain
TYPES
Cerebellar Astrocytomas
• Most common Type
• Benign, Cystic, Slow-growing
• 95% cure rate with surgical excision
TYPES CONT.
Medulloblastomas
• Fast growing
• Highly malignant
• Cerebellum most common site
• 40% survival rate
TYPES CONT.
Brainstem Gliomas
• Slow growing
• astrocytomas or glioblastomas
• Radiation may shrink tumor
TYPES CONT.
Ependymomas
• Varying speed of growth
• Freq. occur in the 4th Ventricle which leads
to CSF Obstruction
• Close to vital centers
• Chemotherapy and radiation
NEUROBLASTOMA
• Definition - Embryonal Tumor of neural
crest cells that normally give rise to the
sympathetic ganglia and adrenal medulla
• Tumor of young children
• Silent tumor - 70% have metastatic disease
at diagnosis
• Primary Site - Addomen
RETINOBLASTOMA
• Definition - Rare congenital tumor that
originates in the retina of one or both eyes
• 200 children a year in the US
• 2 forms - Inherited - Diagnosed during first
year and often involves both eyes, and
Aquired - Diagnosed 2-3 years and 60%
unilateral
RETINOBLASTOMA CONT.
• Primary Sign - LEUKOKORIA, a white
pupillary reflex called “cat’s eye reflex”.
Others - strabismus, red, painful eye, and
limited vision
• Treatment - radiation for small tumors,
large or multiple tumors require
enucleation
• Prognosis - 90% long term survival rate
WILMS TUMOR
• Definition - Embryonal tumor of the kidney,
Nephroblastoma
• Incidence - 7.8 cases per 1 million children.
Approximately 400 children diagnosed each
year or 1 per 10,000 children
• Pathogenesis - 2 forms - Sporadic - No known
genetic predisposition and Inherited
-Autosomal Dominant Transmission
WILMS TUMOR CONT.
• Both forms linked to the deletion or
inactivation of genes on the short arm of
Chromosome #11
• 18% of children with Wilms have other
congenital anomalies
WILMS CONT.
• Clinical Manifestations - 90% have
enlarging asymptomatic upper abdominal
mass that is firm, nontender, smooth, and
encapsulated
• Treatment - Nephrectomy. Radiation
(except in Stage I & II ), Chemotherapy.
• Prognosis - 95% cure rate for Stage I to III
RHABDOMYOSARCOMA
• Definition - Soft tissue sarcoma that arises
from undifferentiated mesenchymal cells
in muscle, tendons, bursae, and fascia, or
fibrous, connective, lymphatic or vascular
tissue
• Two thirds diagnosed by 10 years of age
• Many sites - head and neck most common
RHABDOMYOSARCOMA
• Treatment - Combination surgery, radiation
and chemotherapy
• Prognosis - Long term survival rate 70-80%
NURSING CARE OF CHILD
WITH LEUKEMIA
Prepare Child & Family for Diagnostic and
Therapeutic Procedures
• Bone Marrow Aspiration (W & W -pg. 1252)
• Lumbar Puncture (W & W - pg. 1251)
• Placement of Central Catheter (W& W - pg. 1308-1311)
Boviac
Hickman
Infus-A-Port
PREVENT COMPLICATIONS OF
MYELOSUPPRESSION
• Infection
Goal is prevention
• Hemorrhage
Platelet Count less than 20,000/mm
Epistaxis and Gingival
• Anemia
Transfusions
MANAGE PROBLEMS OF
IREADIATION AND DRUG THERAPY
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Nausea and Vomiting
Anorexia
Mucosal Ulceration
Neuropathy
Hemorrhagic Cystitis
Alopecia
Moon Face
RELIVE PAIN
• Analgesics
• Positioning
• Decrease Environmental Stimuli
PROVIDE ADEQUATE
FAMILY SUPPORT
• Explain tests and procedures honestly
• Schedule uninterrupted family time
• Encourage family to discuss feelings and
concerns
• Encourage active family participation in
care