Pediatric Oncology

Why are we discussing pediatric oncology?

Childhood cancer accounts for only 2% of all cancer cases Childhood cancer has an overall survival rate of 80%
Acute Lymphocytic Leukemia (ALL) is over 90%

HOWEVER:
Cancer is still the leading cause of death in children from disease
Second to accidents

Diagnosis is a life-changing one for the patient and all members of the family
Fear of the unknown difficult to overcome and always in the background

Causes
Cancer by itself needs to be viewed as an assortment of genetic diseases
Not to be confused with hereditary

Cause is usually not determined Most view cancer etiology as multifactorial Types of cell damage
Conversion of proto-oncogenes (regulate cell growth) into oncogenes (abnormal growth) Inactivation of tumor suppressor genes Bypass of DNA repair gene that tells cell to die after damaged Two-hit model

Acquired Capabilities of Cancer

Essentials of Pediatric Oncology Nursing: A Core Curriculum, 2nd Ed. (2004) p. 16

Characteristics of Childhood Ca Vs. Cancer in Adults Childhood

Rare 1-2% of all cases Tissues CNS, muscle, bone, reticuloendothelial system Brief latency Genetic alterations a major role Minimal opportunity for prevention Small opportunity for screening

Adults
98-99% of all cases Organs-breast, lungs, colon, prostate Latency may be 20+ years Environmental & lifestyle factors more than genetics 80% estimated to be preventable Many can be detected by screening methods

Essentials of Pediatric Oncology Nursing: A Core Curriculum, 2nd Ed. (2004) p. 12

Characteristics (cont.)
Childhood
Metastatic in 80% of cases Pharmacokinetics
Markedly different in children because of rapid developmental changes Many common tumor types responsive to chemo May tolerate higher doses & less difficulty with acute toxicity Vulnerable to long-term effects

Adults
Local or regional disease Pharmacokinetics
Common tumor types less responsive to available chemo May have more difficulty with acute toxicity Less issues with long-term effects

Genetic testing very rare Treatment per research protocols very common Prognosis 70-90% cure

Genetic testing more common where available Treatment per research protocols less common Prognosis <60% cure

Essentials of Pediatric Oncology Nursing: A Core Curriculum, 2nd Ed. (2004) p. 12

7 Cardinal Signs of Ca in Children

These signs can also occur in nonmalignant conditions
Presence of a mass Purpura Pallor Weight loss Whitish reflex in the eye Vomiting in the early AM Recurrent or persistent fever

5 More covert signs/symptoms

These also can easily occur in nonmalignant conditions
Bone pain
Increased concern if pain awakens child at night

Headache
Especially in the morning upon awakening

Persistent lymphadenopathy Change in balance, gait, personality Fatigue, malaise

Diagnostic Tests
CBC with diff CMP LDH Alkaline Phosphate Uric acid Alpha fetoprotein Cathecholamines (urine) Cytogenetic studies Chest x-ray Bone marrow
Aspirate and biopsy

CT Scans MRI PET Scan Bone Scan Lumbar Puncture Biopsies

These tests are disease/symptom specific

Treatment for Childhood Cancer

Three major types of treatment
Surgery
Can occur at different points of treatment plan Biopsies Staging Debulking Surgery due to complications of illness Palliation Placement of venous access devices

Treatment of Childhood Ca (cont.)

Chemotherapy
Can occur by itself or with surgery and/or radiation Goals
Reduce tumor volume & prevent tumor cell division & spread Designed to kill tumor cells during different phases of cell cycle Given as combination agents to thus attack cells during different stages at the same time Goals can be Cure, control, and/or palliation

Roles
Multimodal, Neoadjuvant, Adjuvant, Sanctuary, Palliative

Modes of delivery
IV, PO, IM, IT

Treatment of Childhood Ca (cont.)

Radiation Therapy (RT)
Goal is to target the tumor while sparing the surrounding tissues Causes single or double strand DNA breaks Important to maintain Hgb >10
Hypoxic cells are more resistant to RT

Many different methods of delivery Not used in all types of childhood ca

Leukemia
Is a neoplastic disease that involves the bloodforming tissues of the bone marrow, spleen, and lymph nodes During blood cell production the white blood cell precursors proliferate in an abnormal, uncontrolled, and destructive manner
These immature cells crowd into all available spaces Production of normal cells is limited due to lack of space and nutrients

Leukemia (cont.)
The type of leukemia will depend on what cell line is affected and the level of cellular differentiation
ALL Acute Lymphocytic leukemia
75-80%

AML Acute Myelogenous Leukemia

2-25%

CML Chronic Myelocytic leukemia

Less than 5%

Cytogenetic abnormalities are found in more than 90% of patients with ALL
Number of chromosomes and structure

Presenting Signs/Symptoms
Fever Anemia
Malaise, fatigue, pallor

Thrombocytopenia
Bruises, petechiae, bleeding

Hepatosplenomegaly Bone pain (23% of cases) Lymphadenopathy CNS disease (less than 10% of cases at diagnosis)
Headache, vomiting, visual disturbances

Treatment
Varies with diagnosis and risk group AML receives induction chemotherapy to clear marrow and put patient into remission (marrow clear of blasts cells) Frequently these patients will require an allogenic stem cell transplant if a matched related donor is available CNS prophylaxis is given to prevent CNS relapse
Treated even if CNS is clean at diagnosis

Treatment for ALL

Divided into 3 phases Induction (first month of therapy)
Goal is to eliminate as many leukemia cells in marrow as possible During beginning of this period patients are at risk for tumor lysis syndrome Includes CNS treatment along with combination chemotherapy (IV, PO, IM)

Consolidation (usually for around 1 year)

Includes CNS treatment along with chemotherapy Requires more hospital admissions for chemo

Treatment for ALL (cont.)

Maintenance (usually for 2-3 years) Goals are:
Maintain remission Prevent drug resistance from developing Minimize long-term side effects

Includes CNS treatment along with combination chemotherapy (PO, IM, IV) Recurrent disease
Treatment will vary depending on when relapse occurred

Leukemia Case Study

4 year old male with one week history of low grade fevers and fatigue Parents brought child to primary MD this morning due to bruises they noticed CBC done in the office lab showed peripheral blasts Child and parents sent to tertiary hospital for admission and consult with oncology Over the next 24-72 hrs diagnosis is made, bone marrow & LP performed, central line is placed and treatment is begun

Audience Participation Time

What types of issues would you the nurse have to address/deal with while caring for this patient/family? What important nursing routines need to occur?

Nursing Care
Emotional support Strict I & Os Monitor labs Blood products as needed Assist with procedures Monitor for side effects Pain relief

Education
Illness Treatment Care at home Effects on other family members

Administer chemo & other medications HANDWASHING!!

REMEMBER- Care involves more than just the patient, includes parents, siblings, and possibly extended family members. Lifechanging diagnosis with possible fatal outcome. Fear constantly in the background, lots of new information to absorb and grasp.

Osteosarcoma
Malignant tumor of the bone Occurs earlier in females
Due to growth spurt
Males Females

More common in males Usually affects long bones Predominately during 2nd decade of life

60 50 40 30 20 10 0 10 20 30 40 %

Presenting Signs/Symptoms
Pain
Dull, aching, constant Worse at night Frequently attributed to trauma

Swelling of soft tissue (possible) Altered gait (possible) 20% have visible metastatic disease (pulmonary) at initial diagnosis

Treatment
Combination chemotherapy Surgery
Limb salvage or amputation

Continued chemotherapy Usually at least one year for entire course Patients need to be cautioned to be careful with the affected limb prior to surgery
Pathologic fracture always a possibility

If pulmonary mets present may need thoracotomy performed in the future

 15 year old male has sports physical performed and MD noted small amount of swelling on distal femur and pt c/o of increased pain while sleeping X-ray obtained and mass noted Referred to orthopedic surgeon at tertiary center
Biopsy performed and pt diagnosed with osteosarcoma and referred to oncology Diagnostic tests performed as an outpatient Pt admitted 1 week later SDA s/p line placement and will start chemotherapy this afternoon

Osteosarcoma Case Study

Audience Participation Time Again!

What differences do you see in this case study compared to leukemia case study? Anything different in terms of nursing care?

Ewings Sarcoma
Bone tumor
RADIOSENSITIVE

May also develop in soft tissues Small, round, blue-cell tumor Translocation t (11;22) Caucasian (non-Hispanic) or Hispanic
Very rare in Asian or African descent

Presenting Signs/Symptoms
Pain
Intermittent, present for 3-9 months

Palpable mass Pathological fractures more common Fever may be present

Treatment
Basically the same as osteosarcoma although the chemotherapy agents are different
Still chemo, surgery, more chemo

Possible radiation therapy

Usually used if patient has reoccurrence

Brain Tumors
Most common solid tumor in childhood ca
Astrocytomas (also called Gliomas)
50% of all pediatric brain tumors Malignant or benign depending on histology Can occur in a variety of areas of the brain and/or spine

Medulloblastoma
25% of all pediatric brain tumors Most common malignant CNS tumor Usually in cerebellum or 4th ventricle
Hydrocephalus often occurs

Ependymoma (10% of all cases)

Presenting Signs/Symptoms
Symptoms vary depending on location of tumor Sudden onset of symptoms more common in aggressive tumors Types of symptoms
Headache (especially upon awakening in morning) Vomiting Ataxia Nystagmus Cranial nerve deficits Visual changes Seizures

Treatment
Surgery
Goal is maximum resection of tumor
Frequently seen by neurosurgeon before oncologist

Radiation therapy
Delayed in children <3yrs of age

Chemotherapy
Used to delay radiation in younger children

High dose chemotherapy followed by autologous stem cell transplant

Highly malignant tumors with minimal residual disease

Neuroblastoma
Most common extracranial solid tumor in children (8-10%) Develops from neural crest cells
Anywhere along the sympathetic nervous system
Chest, abdomen (may cross the midline), adrenal glands, head, pelvis

Most common malignancy in infants

Diagnosis at < 1yr best prognosis Phenomenon of spontaneous tumor regression & maturation of the tumor can occur in this age group

Neuroblastoma (cont.)
Usually diagnosed between age 2-5 silent tumor
Widespread metastatic disease in 70% of pts at diagnosis
Sites include
Lymph nodes Bone Bone marrow Liver Subcutaneous tissue Hardened bluish nodules

Rarely spreads to lung and brain

Presenting Signs/Symptoms
Pain Abdominal mass Malaise Cathecholamine secretion
HTN, flushing, excessive sweating, irritability

Unique paraneoplastic syndrome (seen in 4% of pts)

Opsomyoclonus
Myoclonic jerking and random eye movements

Cerebellar ataxia

Treatment
Treatment & prognosis depend on staging and age of child Includes:
Surgery Chemo Radiation Stem cell transplant

 Hodgkins & Non-Hodgkins Lymphoma (NHL) NHL

Malignant solid tumor of the immune system involving lymphoid cells Is a generalized disease at presentation Disease spreads in a random, diffuse, unpredictable, and aggressive manner
Quick diagnosis and rapid initiation of treatment is crucial

Lymphoma

Responds rapidly to chemo so need to be very watchful for tumor lysis syndrome Can involve T & B cell lines
Different subdivisions based on cell line involved

Presenting Signs/Symptoms
Vary with site of disease
Abdomen
Pain, N/V, change in bowel habits, distension

Mediastinum
Dysphagia, cough, wheeze, strider, superior vena cava syndrome

Head and Neck

Cervical lymphadenopathy, jaw swelling, unilateral tonsillar enlargement

Bone marrow
Pallor, anemia, thrombocytopenia

Hodgkins
A specific type of lymphoma that involves the spleen and lymphatic system Characterized by binucleate or multinucleated giant cells 5% of all childhood cancers 3 distinct forms
Childhood (14 yr and younger) Young adult (15-34) Older adult (55-74)

Presenting Signs/Symptoms
Painless supraclavicular or cervical adenopathy 2/3 of pts have mediastinal involvement
Coughing, s/s of airway obstruction

Fatigue Anorexia 3 constitutional B symptoms

(correlate with prognosis) Unexplained fever above 38 C Unexplained weight loss (10% within 6 months) Drenching night sweats

Treatment
Treatment
Either or both:
Chemotherapy Radiation therapy

Children & adolescents in the favorable prognostic group have a 95% cure rate

Retinoblastoma
Intraocular tumor
80% pts are under age 4 yrs Familial form in 40% of cases
Predisposed affected patient to secondary neoplasms

Presentation
Leukocoria (cats eye reflex)
Lack of normal red reflex of the eye

Strabismus Esotropia or exotropia Decreased vision in one eye

Treatment
Treatment (one or more of these therapies)
Enucleation Radiation Cryotherapy or thermotherapy Chemotherapy Laser photocoagulation

Wilms Tumor
Tumor of the kidney 6% of all childhood cancers Familial cases 1-2% Presentation
Asymptomatic abdominal mass (20-30% of pts)
pain Malaise Hematuria HTN

Treatment
Surgery Chemotherapy
Based on staging Possibly prior to surgery

Radiation therapy
Based on staging

80-more than 90% cure rate including stage IV disease

 Tumors that arise from cells of skeletal muscle lineage

Can occur in tissues in which striated muscle cells are not normally found

Rhabdomyosarcoma

Presentation
Mass or disturbance of normal body function
Head and neck including orbits (35%) Genitourinary tract (22%) Extremities (18%) Other sites (25%)
Trunk, pelvis, retroperitoneal, biliary tract, liver, brain, trachea, heart, breast, ovary

General Nursing Care

Varies with what stage of illness is occurring REMEMBER THIS IS A CHRONIC DISEASE WITH ACUTE PERIODS Teaching and reinforcement of teaching is common thread to all stages of illness

Newly Diagnosed
Many questions Need for emotional support Lots of important decisions to be made
In a fairly short period of time

May want second opinion Lots of teaching Nursing needs

I&O Blood products -Surgical post-op needs -Reverse isolation

Treatment
Frequent admissions
Weekly to q 3 weeks is norm

I&O Large amount of hydration Chemo administration Monitor labs Supportive management
Nausea and vomiting

Reinforce teaching

Neutropenia other non-scheduled admits

Neutropenia is the #1 dose-limiting symptom for chemotherapy Emergency admission
Can occur 7-10 days after chemo txment

High risk for sepsis Close monitoring of v/s and labs Antibiotics -I & O -Supportive management
Pain, mucositis, fatigue

End of Treatment
Happy to be over with treatments but fearful at same time to have them end
Support system from staff may be less Fear of return of cancer Change in routines
Especially for younger patients

Reoccurrence
No longer nave-fully aware of what is involved Body tired from previous chemotherapy cycle
May develop side effects quicker and/or more severely

Reality that cure is less of a possibility

End Stage
Acceptance of terminal stage may or may not have occurred Could be giving supportive care only May still be giving active treatment

Survivorship Issues
Long term effects to major organ systems
Renal, cardiac Can occur many years after therapy completed

Genetic issues Hearing impairments Hepatitis C Infertility and sexuality issues Insurance

Survivorship Issues (cont.)

Learning disabilities Limb loss Secondary cancers Importance of yearly follow-ups with practitioners that are knowledgeable of survivorship issues!!

 Life-changing diagnosis that affects the entire family Patient may be medical or surgical at different times in their treatment course Treatment courses can last for 1-3 years of more if relapses occur Fear in the background even if not obvious Try to encourage families to maintain normalcy as much as possible
Home, school, friends

Balance your involvement!