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CANCER IN CHILDREN

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 39.6* 35.7 22.1 Lymphoma 03.7 13.3 16.4* CNS 15.0 27.1* 18.9 Wilms Tumor 09.2* 06.1 02.0 Neuroblastoma 13.0* 02.2 00.5

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CANCER IN CHILDREN

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  1. CANCER IN CHILDREN KAREN E. MILES FLORIDA GULF COAST UNIVERSITY DEPARTMENT OF NURSING

  2. CHILDHOOD CANCERS BY AGE SITE 0-5 YR 5-10 YR 10-15 YR Leukemia 39.6* 35.7 22.1 Lymphoma 03.7 13.3 16.4* CNS 15.0 27.1* 18.9 Wilms Tumor 09.2* 06.1 02.0 Neuroblastoma 13.0* 02.2 00.5 Retinoblastoma 06.4* 01.3 00.0 Bone 00.6 05.0 10.5* Other 08.8 05.8 23.6 *Peak Incidence

  3. INCIDENCE • 14 cases per 100,000 children/yr • 10-25% more common in white than black children

  4. ETIOLOGY • Largely unknown • Most likely - complex interactions of both genetic and environmental factors - Ecogentics

  5. 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)

  6. 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)

  7. 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

  8. PROGNOSIS • 70% will be cured • Children are more responsive to tx and better able to tolerate immediate side effects of surgery

  9. 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)

  10. 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

  11. 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

  12. CLASSIFICATION CONT. Immunologic Classification - based on certain 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)

  13. BEST PROGNOSIS • ALL 1 • Early Pre B- Cell • CALLA Positive

  14. 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

  15. 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

  16. CLINICAL MANIF. CONT. • Infiltration into bones and joints • Infiltration of other organ sites: kidneys, heart, eyes, skill and GI tract

  17. 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

  18. 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

  19. PROGNOSIS • ALL IS CURABLE • Overall - 60-70% • ALL1, Pre- B Cell, CALLA Positive - 90%

  20. 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

  21. LYMPHOMAS CONT. • Most frequent site - Abdomen • Treatment - Biopsy, combination chemotherapy and radiation • 60-80% can be cured

  22. 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

  23. 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

  24. TYPES Cerebellar Astrocytomas • Most common Type • Benign, Cystic, Slow-growing • 95% cure rate with surgical excision

  25. TYPES CONT. Medulloblastomas • Fast growing • Highly malignant • Cerebellum most common site • 40% survival rate

  26. TYPES CONT. Brainstem Gliomas • Slow growing • astrocytomas or glioblastomas • Radiation may shrink tumor

  27. 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

  28. 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

  29. 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

  30. 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

  31. 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

  32. 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

  33. 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

  34. 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

  35. RHABDOMYOSARCOMA • Treatment - Combination surgery, radiation and chemotherapy • Prognosis - Long term survival rate 70-80%

  36. 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) Boviac Hickman Infus-A-Port

  37. PREVENT COMPLICATIONS OF MYELOSUPPRESSION • Infection Goal is prevention • Hemorrhage Platelet Count less than 20,000/mm Epistaxis and Gingival • Anemia Transfusions

  38. MANAGE PROBLEMS OF IREADIATION AND DRUG THERAPY • Nausea and Vomiting • Anorexia • Mucosal Ulceration • Neuropathy • Hemorrhagic Cystitis • Alopecia • Moon Face

  39. RELIVE PAIN • Analgesics • Positioning • Decrease Environmental Stimuli

  40. 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

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