PEDIATRIC ONCOLOGY


Leukemia

  1. General information
    1. Most common form of childhood cancer
    2. Peak incidence is 3 to 5 years of age
    3. Proliferation of abnormal white blood cells that do not mature beyond the blast phase
    4. In the bone marrow, blast cells crowd out healthy white blood cells, red blood cells, and platelets, leading to bone marrow depression
    5. Blast cells also infiltrate other organs, most commonly the liver, spleen, kidneys, and lymph tissue
    6. Symptoms reflect bone marrow failure and associated involvement of other organs
    7. Types of leukemia, based on course of disease and cell morphology
      1. Acute lymphocytic leukemia (ALL)
        1. 80-85% of childhood leukemia
        2. malignant change in the lymphocyte or its precursors
        3. acute onset
        4. 95% chance of obtaining remission with treatment
        5. 75% chance of surviving 5 years or more
        6. prognostic indicators include: initial white blood count (less than 10,000/mm3), child's age (2-9 years), histologic type, sex
      2. Acute nonlymphocytic leukemia (ANLL)
        1. includes granulocytic and monocytic types
        2. 60-80% will obtain remission with treatment
        3. 30-40% cure rate
        4. prognostic indicators less clearly defined
  2. Medical management
    1. Diagnosis: blood studies, bone marrow biopsy
    2. Treatment stages
      1. Induction: intense and potentially life threatening
      2. CNS prophylaxis: to prevent central nervous system disease. Combination of radiation and intrathecal chemotherapy.
      3. Maintenance: chemotherapy for 2 to 3 years.
  3. Assessment findings
    1. Anemia (due to decreased production of RBCs), weakness, pallor, dyspnea
    2. Bleeding (due to decreased platelet production), petechiae, spontaneous bleeding, ecchymoses
    3. Infection (due to decreased WBC production), fever, malaise
    4. Enlarged lymph nodes
    5. Enlarged spleen and liver
    6. Abdominal pain with weight loss and anorexia
    7. Bone pain due to expansion of marrow
  4. Nursing interventions
    1. Provide care for the child receiving chemotherapy and radiation therapy.
    2. Provide support for child/family; needs will change as treatment progresses.
    3. Support child during painful procedures (frequent bone marrow aspirations, lumbar punctures, venipunctures needed).
      1. Use distraction, guided imagery.
      2. Allow child to retain as much control as possible.
      3. Administer sedation prior to procedure as ordered.


Brain Tumors

  1. General information
    1. A space-occupying mass in the brain tissue; may be benign or malignant
    2. Males affected more often; peak age 3-7 years
    3. Second most prevalent type of cancer in children
    4. Cause unknown; genetic and environmental factors may play a role; familial tendency for brain tumors, which are found with preexisting neurocutaneous disorders.
    5. Two-thirds of all pediatric brain tumors are beneath the tentorium cerebelli (in the posterior fossa), often involving the cerebellum or brain stem.
    6. Three-fourths of brain tumors in children are gliomas (medulloblastoma and astrocytoma).
  2. Types
    1. Medulloblastoma: highly malignant tumor usually found in cerebellum; runs a rapid course
      1. Findings include increased ICP plus unsteady walk, ataxia, anorexia, early morning vomiting
      2. Treated with radiation since complete removal is impossible
    2. Astrocytoma: a benign, cystic, slow-growing tumor usually found in cerebellum
      1. Onset of symptoms is insidious.
      2. Findings include focal disturbances, papilledema, optic nerve atrophy, blindness.
    3. Ependymoma: a usually benign tumor that arises in the ventricles of the brain, causing noncommunicating hydrocephalus and damage (by pressure) to other vital tissues of the brain
    4. Craniopharyngioma: tumor that arises from remnants of embryonic tissue near the pituitary gland in the sella turcica, causes pressure on the third ventricle
      1. Decreased secretion of ADH causes diabetes insipidus (these children may need Pitressin).
      2. Additional symptoms include altered growth pattern, visual difficulties, difficulty regulating body temperature.
    5. Brain stem glioma: slow-growing tumor, indicated by cranial nerve palsies, ataxia
  3. Medical management
    1. Surgery: some tumors entirely or partially resected; others are not amenable to surgery because of proximity to vital brain parts
    2. Radiation therapy: often used to shrink tumors
    3. Chemotherapy: vincristine, lomustine, procarbazine, intrathecal methotrexate; not as effective with brain tumors as with other childhood cancers
  4. Assessment findings
    1. Symptoms dependent on location and type of tumor.
    2. A definite diagnosis is difficult in children because of the elasticity of child's skull and generally poor coordination of the young child.
    3. A decrease in school performance may be the first sign.
    4. Increased ICP
      1. Morning headache
      2. Morning vomiting without nausea; vomiting without relation to feeding schedule; projectile vomiting
      3. Personality changes
      4. Diplopia
        1. difficult to assess in young children
        2. observe child for tilting of head, closing or covering one eye, rubbing the eyes, or impaired eye-hand coordination
      5. Papilledema: a late sign
      6. Increased blood pressure with decreased pulse: also a late sign
      7. Cranial enlargement
        1. more readily noticeable prior to 18 months when suture lines are still open
        2. bulging, tense, pulsating fontanels
        3. widened suture lines
        4. 90% or more on head circumference chart
    5. Focal signs and symptoms
      1. Ataxia
        1. in cerebellar tumors
        2. may not be readily identified because of uncoordinated movements of young children
      2. Muscle strength
        1. weakness with cerebellar tumors
        2. weakness, spasticity, and paralysis of lower extremities with cerebral or brain stem tumors
        3. change in handedness, posture, or manual coordination: may be early signs
      3. Head tilt
        1. in posterior fossa tumors
        2. early sign of visual impairment
        3. associated with nuchal rigidity
        4. due to traction on the dura
      4. Ocular signs
        1. nystagmus: corresponds to the same side as the infratentorial lesion
        2. diplopia/strabismus: from palsy of cranial nerve VI with brain stem glioma or increased ICP
        3. visual field deficit (child does not react to activity on periphery of vision): with craniopharyngiomas
      5. Seizures: with cerebral tumors
    6. Diagnostic tests
      1. Skull x-ray reveals presence and location of tumor
      2. CT scan (with or without contrast dye) reveals position, consistency, size of tumor, and effect on surrounding tissue
      3. EEG may show seizure activity
  5. Nursing interventions
    1. Obtain baseline vital signs and perform thorough neurologic assessment; monitor vital signs and neurologic status frequently.
    2. Prevent injury/complications.
      1. Institute seizure precautions.
      2. Monitor for fluid and electrolyte imbalance from vomiting.
      3. Observe for increased ICP.
      4. Provide safety measures (bed rails up).
    3. Promote comfort/relief of headache.
      1. Decrease environmental stimuli.
      2. Administer analgesics as ordered.
    4. Prevent constipation (straining increases ICP).
      1. Provide appropriate foods and fluids as ordered.
      2. Provide stool softeners as ordered.
      3. Avoid enemas, which increase ICP.
    5. Provide care for the child undergoing brain surgery.
    6. Provide care for the child undergoing radiation or chemotherapy.
    7. Provide client teaching and discharge planning concerning
      1. Diagnostic tests (instruction needs to be appropriate to the child's developmental level)
        1. machines will make clicking sounds
        2. wires attached to the head for an EEG will not electrocute child
        3. head is immobilized for a CT scan
        4. the use of contrast dye and expected sensations if used
        5. need to lie still with the technician out of the room for most tests (younger children will be sedated for fuller cooperation)
      2. Importance of family discussion of fears/ anxiety about surgery and prognosis
      3. Need to assist in implementing child's interaction with peers
      4. Available support groups and community agencies


Brain Surgery

  1. General information
    1. Indications
      1. Removal of a tumor
      2. Evacuation of a hematoma
      3. Removal of a foreign body or skull fragments resulting from trauma
      4. Aspiration of an abscess
      5. Insertion of a shunt
  2. Nursing interventions: preoperative
    1. Assess the child's understanding of the procedure; have the child draw a picture, tell a story; observe doll play.
    2. Explain the procedure in terms according to the child's developmental level.
    3. Allow the child to visit the operating room/intensive care unit, if permitted, depending on the child's emotional and developmental levels
    4. Explain that pre-op symptoms such as headache and ataxia may be temporarily aggravated.
    5. Advise child/parents that blindness may result, depending on the location of the tumor.
    6. Inform the child/parents that the head will be shaved; long hair may be saved; hats or scarves may be used to cover the head once the dressings are removed.
    7. Support the child/family if a tumor cannot be totally removed.
    8. Provide instruction about radiation and chemotherapy (may need to be delayed since detail may be overwhelming).
    9. Explain to the child/parents about the post-op dressing, monitoring devices, and possibility of facial edema.
  3. Nursing interventions: postoperative
    1. Prevent injury/complications.
      1. Monitor vital signs and neuro status frequently until stable.
      2. Apply hypothermia blanket as ordered.
      3. Assess respiratory status/signs of infection.
      4. Observe the dressing for discharge/hemorrhage.
      5. Close or cover eyes, apply ice, instill saline drops or artificial tears.
      6. Position as ordered according to the location of the tumor and type of surgery.
      7. Assess for increased ICP.
      8. Institute seizure precautions.
    2. Promote comfort.
      1. Decrease environmental stimuli.
      2. Administer analgesics as ordered, first assessing LOC.
    3. Promote adequate nutrition.
      1. Administer fluids as ordered.
      2. Monitor I&O.
      3. Provide diet as ordered.
    4. Provide emotional support and encourage child/family to discuss prognosis.
    5. Provide client teaching and discharge planning concerning
      1. Wound care
      2. Signs of increased ICP
      3. Activity level
      4. Sensation and time period of hair growth
      5. Peer acceptance
      6. Radiation/chemotherapy, if indicated
      7. Availability of support groups/community agencies


Hodgkin's Lymphoma

  1. General information
    1. Malignant neoplasm of lymphoid tissue, usually originating in localized group of lymph nodes; a proliferation of lymphocytes
    2. Metastasizes first to adjacent lymph nodes
    3. Cause unknown
    4. Most prevalent in adolescents; accounts for 5% of all malignancies
    5. Prognosis now greatly improved for these children; influenced by stage of disease and histologic type
    6. Long-term treatment effects include increased incidence of second malignancies, especially leukemia and infertility
  2. Medical management
    1. Diagnosis: extensive testing to determine stage, which dictates treatment modality
      1. Lymphangiogram determines involvement of all lymph nodes (reliable in 90% of clients); is helpful in determining radiation fields
      2. Staging via laparotomy and biopsy
        1. stage I: single lymph node involved; usually in neck; 90%-98% survival
        2. stage II: involvement of 2 or more lymph nodes on same side of diaphragm; 70%-80% survival
        3. stage III: involvement of nodes on both sides of diaphragm; 50% survival
        4. stage IV: metastasis to other organs
      3. Laparotomy and splenectomy
      4. Lymph node biopsy to identify presence of Reed-Sternberg cells and for histologic classification
    2. Radiation: used alone for localized disease
    3. Chemotherapy: used in conjunction with radiation therapy for advanced disease
  3. Assessment findings
    1. Major presenting symptom is enlarged nodes in lower cervical region; nodes are nontender, firm, and movable
    2. Recurrent, intermittent fever
    3. Night sweats
    4. Weight loss, malaise, lethargy
    5. Pruritus
    6. Diagnostic test: presence of Reed-Sternberg cells
  4. Nursing interventions
    1. Provide care for child receiving radiation therapy.
    2. Administer chemotherapy as ordered and monitor/alleviate side effects.
    3. Protect client from infection, especially if splenectomy performed.
    4. Provide support for child/parents; specific needs of adolescent client must be considered.


Non-Hodgkin's Lymphoma

  1. General information
    1. Tumor originating in lymphatic tissue
    2. Significantly different from Hodgkin's lymphoma
      1. Control of primary tumor is difficult
      2. Disease is diffuse, cell type undifferentiated
      3. Tumor disseminates early
      4. Includes wide range of disease entities: lymphosarcoma, reticulum cell sarcoma, Burkitt's lymphoma
    3. Primary sites include GI tract, ovaries, testes, bone, CNS, liver, breast, subcutaneous tissues
    4. Affects all age groups.
  2. Medical management
    1. Chemotherapy: multiagent regimens including cyclophosphamide (Cytoxan), vincristine, prednisone, procarbazine, doxorubicin, bleomycin
    2. Radiation therapy: primary treatment in localized disease
    3. Surgery for diagnosis and clinical staging
  3. Assessment findings
    1. Depend on anatomic site and extent of involvement
    2. Rapid onset and progression
    3. Many have advanced disease at diagnosis
  4. Nursing interventions: provide care for child receiving chemotherapy, radiation therapy, and surgery.


Wilm's Tumor (Nephroblastoma)

  1. General information
    1. Large, encapsulated tumor that develops in the renal parenchyma, more frequently in left kidney (usually unilateral)
    2. Originates during fetal life from undifferentiated embryonic tissues
    3. Peak age of occurrence: 1-3 years
    4. Prognosis good if there are no metastases.
  2. Medical management
    1. Nephrectomy, with total removal of tumor
    2. Postsurgical radiation in treatment of stages II, III, and IV; stage I disease does not usually require radiation, but it may be used if the tumor histology is unfavorable.
    3. Postsurgical chemotherapy: vincristine and daunorubicin, doxorubicin
  3. Assessment findings
    1. Staging
      1. Stage I: limited to kidney
      2. Stage II: tumor extends beyond kidney, but is completely encapsulated
      3. Stage III: tumor confined to abdomen
      4. Stage IV: tumor has metastasized to lung, liver, bone, or brain
      5. Stage V: bilateral renal involvement at diagnosis
    2. Usually mother notices mass while bathing or dressing child; nontender, usually midline near liver
    3. Hypertension and possible hematuria, anemia, and signs of metastasis
    4. Diagnostic test: IVP reveals mass
  4. Nursing interventions
    1. Do not palpate abdomen to avoid possible dissemination of cancer cells.
    2. Handle child carefully when bathing and giving care.
    3. Provide care for the client with a nephrectomy; usually performed within 24-48 hours of diagnosis.
    4. Provide care for the child receiving chemotherapy and radiation therapy.


Neuroblastoma

  1. General information
    1. A highly malignant tumor that develops from embryonic neural crest tissue; arises anywhere along the craniospinal axis, usually from the adrenal gland
    2. Incidence
      1. One in 10,000
      2. Males slightly more affected
      3. From infancy to age 4
    3. Staging
      1. Stage I: tumor confined to the organ of origin
      2. Stage II: tumor extends beyond primary site but not across midline
      3. Stage III: tumor extends beyond midline
      4. Stage IV: tumor metastasizes to skeleton (bone marrow), soft tissue (liver), and lymph nodes
  2. Medical management: depends on the staging of tumor and age of child; includes surgery, radiation therapy, chemotherapy
  3. Assessment findings vary, depending on the tumor site and stage
    1. If in the abdomen, may initially resemble Wilm's tumor
    2. Local signs and symptoms caused by pressure of the tumor on surrounding tissue
    3. Metastatic manifestations
      1. Ocular: supraorbital ecchymosis, periorbital edema, exophthalmos
      2. Cervical or supraclavicular lymphadenopathy
      3. Bone pain: may or may not occur with bone metastasis
      4. Nonspecific complaints; pallor, anorexia, weight loss, irritability, weakness
    4. Diagnosis usually made after metastasis has occurred
    5. Diagnostic tests
      1. X-rays of the head, chest, or abdomen reveal presence of primary tumor or metastases
      2. IVP: if tumor is adrenal, shows a downward displacement of the kidney on the affected side
      3. Bone marrow aspiration: to rule out metastasis; neuroblasts have a clumping pattern
      4. CBC: RBCs and platelets decreased
      5. Coagulation studies: abnormal due to thrombocytopenia
      6. Catecholamine excretion: VMA levels in urine increased
        1. child must not ingest vanilla, chocolate, bananas, or nuts for 3 days prior to the test
        2. 24-hour urine specimen needed
  4. Nursing interventions: same as for leukemia (see Pediatric Oncology - Cancers in Unit 5) and brain tumors.


Bone Tumors


Osteogenic Sarcoma

  1. General information
    1. Primary bone tumor arising from the mesenchymal cells and characterized by formation of osteoid (immature bone)
    2. Invades ends of long bones, most frequently distal end of femur or proximal end of tibia
    3. Occurs more often in boys, usually between ages 10-20 years
    4. Lungs most frequent site of metastasis
    5. 5-year survival rate is 10%-20%
  2. Medical management
    1. Surgery: treatment of choice
      1. Amputation: temporary prosthesis used immediately after surgery; permanent one usually fitted a few weeks later
      2. Limb salvage procedures
      3. Lung surgery if there are metastases
    2. Radiation: only in areas where tumor is not accessible to surgery
    3. Chemotherapy: adjuvant therapy being studied
  3. Assessment findings
    1. Insidious pain, increasing with activity, gradually becoming more severe
    2. Tender mass, warm to touch; limitation of movement
    3. Pathologic fractures
  4. Nursing interventions
    1. Prepare child for amputation: discuss fears, concerns, and facts of procedure; answer questions regarding prosthetic devices, limited activity
    2. Assure child that phantom limb pain will subside


Ewing's Sarcoma

  1. General information
    1. Primary tumor arising from cells in bone marrow
    2. Invades bone longitudinally, destroying bone tissue; no new bone formation
    3. Femur most frequently affected site
    4. More common in males, between ages 5-15 years
    5. Lungs most frequent site of metastasis
  2. Medical management
    1. High-dose radiation is primary treatment
    2. Chemotherapy
    3. Value of surgery presently being reassessed
  3. Assessment findings
    1. Pain and swelling
    2. Palpable mass, may be tender, warm to touch
    3. 15%-35% of clients have metastatic disease at time of diagnosis
  4. Nursing interventions
    1. Promote exercise of affected limb to maintain function.
    2. Avoid activities that may cause added stress to affected limb.

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