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