CANCER IN CHILDREN KAREN E. MILES FLORIDA GULF COAST UNIVERSITY DEPARTMENT OF NURSING
Dec 14, 2015
CANCER IN CHILDREN
KAREN E. MILESFLORIDA GULF COAST
UNIVERSITYDEPARTMENT OF NURSING
CHILDHOOD CANCERS BY AGE
SITE 0-5 YR 5-10 YR 10-15 YRLeukemia 39.6* 35.7 22.1Lymphoma 03.7 13.3 16.4*CNS 15.0 27.1* 18.9Wilms Tumor 09.2* 06.1 02.0Neuroblastoma 13.0* 02.2 00.5Retinoblastoma 06.4* 01.3 00.0Bone 00.6 05.0 10.5*Other 08.8 05.8 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 non-white• 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 oncertain surface cell antigens
• T- Cell - common in older boys• 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 siteMOST 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 CHILDWITH 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)
BoviacHickmanInfus-A-Port
PREVENT COMPLICATIONS OF MYELOSUPPRESSION
• InfectionGoal is prevention
• HemorrhagePlatelet Count less than
20,000/mmEpistaxis and Gingival
• AnemiaTransfusions
MANAGE PROBLEMS OF IREADIATION AND DRUG
THERAPY• 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