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pediatric cancer def.
a group of chronic diseases characterized by uncontrolled growth and spread of abnormal cells which, if not treated adequately, results in the death of the child
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what is cancer
- genetic mutation
- permanent DNA alteration
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where does cancer begin
in a single cell
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cancer (the genetic mutation part of it) results in (4)
- lack of differentiation
- loss of contact inhibition
- unregulated growth
- cellular immortal
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cure rate of children vs adults w/ cancer
- children: more than 70% cured
- adults: less than 60% cured
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origin of children vs. adult cancers
- children: primitive embryonic origin
- adults: epithelial origin
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genetic or envtal (children vs adult cancers)
- children: genetically-linked
- adults: stronger link to envt
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cancer screening in adults vs children
- children: no screening
- adults: screening is routine
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prevention in adult vs child cancer
- children: few preventative measures
- adult: many adult cancers are preventable
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metastasis present at dx (adult vs child cancer)
- children: metastatic disease present at diagnosis
- adult: many do NOT present w/ metastasis
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adult vs. child cancer response to treatment
- children: more responsive to treatment
- adult: less responsive to treatment
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national collaborative study group for children's cancers
Children's Oncology Group
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clinical trials are more strict for children. what are 4 things re: the strictness of these trials
- imrove therapy
- decrease morbidity
- increase survival
- stress impt.ance of follow-up
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goal of treatment of child cancer
rid body of ALL malignant cells
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typed of treatment of child cancers: combination? same as adults?
- yes, combination
- yes, same as adults
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5 child cancers
- leukemias (AML and ALL)
- lymphomas (Hodgkin's and Non-Hodgkin's)
- brain tumors (the most common ones)
- neuroblastomas
- Wilms tumors
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desc leukemia
group of malignant diseases of bone marrow and lymphatic system
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#1 cancer in children under 15?
leukemias
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bone marrow physiology of leukemias
normal bone marrow replaced by blast cells, causing marrow to produce immature WBCs
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2 most common types leukemia in children and their survival rates
- Acute Lymphocytic Leukemia (80%)
- Acute Myelogenous Leukemia (40-50%)
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Acute Lymphocytic Leukemia is what % of all child leukemias
80%
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Acute Lymphocytic Leukemia is what % of all child cancers
1/3rd
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peak incidence age of Acute Lymphocytic Leukemia
2-5 yrs
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factors increasing risk of Acute Lymphocytic Leukemia
- virus
- genetic predisposition (Downs, Bloom Immunodeficiency states)
- exposure to toxins (exposure in utero)
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how long are s/s Acute Lymphocytic Leukemia present
1-2 weeks
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3 main s/s leukemia
- fatigue
- fever, infection
- petechiae, bleeding
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how confirm dx of leukemia
bone marrow aspirate
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how treat Acute Lymphocytic Leukemia, and what's the procedure over how long a time
- systemic chemotherapy
- 4 step process
- up to 30 months
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step 1 of 4-step process for Acute Lymphocytic Leukemia treatment: what? how long? goal?
- induction therapy
- 4 weeks
- goal - complete remission
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step 2 of 4-step process for Acute Lymphocytic Leukemia treatment: what? goal?
- CNS prophylactic therapy
- goal - prevent leukemic cells from invading CNS
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step 3 of 4-step process for Acute Lymphocytic Leukemia treatment: what? how long? goal?
- consolidation or intensification therapy (high dose chemo)
- 6 months
- goal - rid any lasting leukemia cells and prevent emerging resistant leukemic clones
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step 4 of 4-step process for Acute Lymphocytic Leukemia treatment: what? how long? goal?
- maintenance
- 17 to 30 months
- goal - maintain remission
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% reach remission in Acute Lymphocytic Leukemia
95%
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% have 5-yr survival in Acute Lymphocytic Leukemia
80%
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if child relapses w/ Acute Lymphocytic Leukemia, prognosis?
prognosis is poor
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w/ Acute Lymphocytic Leukemia, what are 2 NOT recommended courses of treatment during first remission
- stem cell transplant
- bone marrow transplant
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Acute Mylegenous Leukemia makes up what % all child leukemias
15 to 25 %
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Acute Mylegenous Leukemia or ALL have poorer prognosis
Acute Mylegenous Leukemia
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etiology Acute Mylegenous Leukemia
unknown
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s/s Acute Mylegenous Leukemia
- VARY GREATLY
- flu-like symptoms
- hemorrhage
- chloromas
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how dx Acute Mylegenous Leukemia
bone marrow aspirate
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what % of Acute Mylegenous Leukemia cases are malignant myeloid blasts
more than 25%
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2 treatments for Acute Mylegenous Leukemia
- systemic chemotherapy
- bone marrow/stem cell transplant
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systemic chemo for Acute Mylegenous Leukemia may also require what 3 treatments
- platelet transfusion
- FFP (fresh frozen plasma)
- cryoprecipitates (to control bleeding)
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the character of systemic chemo for Acute Mylegenous Leukemia
- intense
- hospitalizaton required
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who is best donor for bone marrow/stem cell transplant for Acute Mylegenous Leukemia pt
sibling
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when do you do a bone marrow/stem cell transplant for Acute Mylegenous Leukemia
once remission is achieved
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chemo and leukemia both cause what, which puts child at risk for infection
- myelosuppression
- implement and edu re: handwashing
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Acute Mylegenous Leukemia - nurse keep in mind what
- infection - handwashing
- bleeding - may need blood prods
- n/v - treat before s/s appear
- anorexia
- mucosal ulceration - pain w/ eat, may need rehydration d/t lack of oral fluids
- alopecia
- emotional support!
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2 types lymphomas and who gets them more
- hodgkins - 15-19 yrs
- nonhodgkins - under 14 yrs
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hodgkins usually originates where
cervical lymph nodes
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etiology hodgkins
may be assoc w/ Epstein-Barr Virus
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hodgkins prevalence boys vs girls
boys more than girls
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hodgkins is what % child malignancies
5%
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hodgkins s/s acute or no?
non acute
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hodgkins s/s
- enlarged, painless cervical nodes (MOST COMMON)
- malaise
- fever
- night sweats
- usually localized at dx
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hodgkins can metastasize to where usually
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how long chemo for hodgkins
6 months, intense
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hodgkins - radiation?
yes, in addition to chemo, radiation to lymph node chain
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w/ adolescents who have hodgkins, as RN, be concerned re: what
sterility, handwashing, infection, etc.
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more common: hodgkins or nonhodgkins
nonhodgkins
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3 types of nonhodgkins
- Burkitts
- Lymphoblastic
- Large B Cell
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nonhodgkins desc.
- disseminated disease
- no single foci of origin
- rapid onset
- widespread environment
- aggressive cancer
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age and boys vs girls: more nonhodgkins
- peak 7 to 11 yrs
- more boys than girls
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nonhodgkins etiology
- viral
- genetic
- immunologic
- environmental
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how does nonhodgkins manifest (what will person c/o)
- w/ pain, swelling
- acute onset
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s/s nonhodgkins
- mediastinal mass
- pleural effusion
- lymphadenopathy
- petechiae
- bruising
- bleeding
- bone pain
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how to dx and stage nonhodgkins
- dx: biopsy of enlarged lymph node
- staging: bone marrow aspirate and biopsy; lumbar puncture (to see CNS involvement)
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how do you check/test for CNS involvement of nonhodgkins
lumbar puncture
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many nonhodgkins are dx'ed at what stages?
stage II or IV
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how treat nonhodgkins
- chemotherapy
- irradiation
- bone marrow transplant (for relapse)
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if nonhodgkins pt relapses, what add'l therapy treatment will you use
Bone marrow transplant
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things nurse should look for in nonhodgkins pt
- infection
- thrombocytopenia
- tumor lysis syndrome
- anorexia (wt loss)
- n/v (wt loss)
- mucositis
- pain
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most common brain tumor and %
astrocytomas 50%
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brain tumor etiology
unknown/unproven
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how are brain tumors classified (2 things)
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brain/CNS tumors are how common
- 2nd most common childhood cancer
- most common solid cancer in children
- 3rd leading cause of death in children under 16
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s/s brain tumor in infants
raised or tense fontanel
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s/s brain tumor
- headache
- vomiting
- hemiparesis
- spastic gait
- seizures
- ataxia
- visual changes
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s/s of brain tumor happen as a result of what
increased ICP
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4 tests to dx brain tumor
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avoid radiation in children w/ brain tumor of what age
less than 3 yrs old
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how treat brain tumor in children
- surgery (remove all or part of tumor)
- radiation to shrink tumor (avoid in less that 3 yrs old)
- chemo (if tumor can't be removed w/ radiation and surgery)
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nurse remember what re: brain tumor children
- neuro checks - observe for s/s increased ICP
- pain control
- post op, avoid increased ICP (watch for vomitting, give steroids, I/O, freq VS)
- ASSESS DEVTAL/BEHAVIORAL ALTERATIONS
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neuroblastomas are how common
- 4th most common tumor in children
- 8-10% all childhood cancers
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why is neuroblastoma called the "silent" tumor
b/c 70% of all cases, metastasis has occurred already by the time of diagnosis
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neuroblastomas originate from where
- ebryonic origins
- neural crest cells of the adrenal medulla, sympathetic ganglia
- "an embryonal malignancy of the sympathetic nervous system arising from neuroblasts (pluripotent sympathetic cells). In the developing embryo, these cells invaginate, migrate along the neuraxis, and populate the sympathetic ganglia, adrenal medulla, and other sites"
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what % of children ar dxed at what age for neuroblastomas
- 39% at less than 1 yr old
- 79% at less than 4 yrs old
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s/s neuroblastomas - remember what
vary according to location of tumor
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most common site for neuroblastoma? most common method of detecting this?
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s/s neuroblastoma (4)
- respiratory (mediastinal tumor)
- edema face/neck (mediastinal tumor)
- altered b/b fx (retroperineal mass)
- pancytopenia (bone marrow involvement)
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test to dx neuroblastoma
tissue biopsy
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tests to stage neuroblastoma
- METASTATIC WORKUP, i.e...
- skeletal survey
- xray of lungs
- bone marrow biopsy
- CT of skull, neck, chest, abd
- abd ultrasound
- urine test measuring vanillylmandelic acid VMA and homovanillic acid HVA
- Int'l Neuroblastoma Staging System
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treatment of neuroblastomas depends mostly on what 2 things
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how treat stage 1 and 2 neuroblastoma
biopsy and resect tumor only
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how treat stage 3 and 4 neuroblastoma
multiagent chemo and radiation
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is bone marrow transplant curative for neuroblastoma
no
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another name for Wilm's tumor
intra-renal tumor
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how common is Wilm's tumor and when are you most likely to get it
- fifth most common cancer
- highest incidence between 2 and 3 yrs old
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etiology Wilm's tumor
genetic link/congenital anomalies
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Wilm's tumors growth rate
- rapidly
- double in size in 11 to 14 days
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Wilm's tumor is d/t what physiologically
- embryonic malformation
- undifferentiated primitive cells
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prognosis for Wilm's tumor
good: 92%
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do you use radiation with Wilm's tumor
only in metastasis, or if residual tumor after surgery, or for recurrence
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what to know re: chemo and Wilm's tumor
may used be pre or post op
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re: Wilm's tumor surgical removal - what do you remove and what should you remember about this surgery
- remove mass and/or kidney
- ctrl hypertension during surgery
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What to remember about palpation and Wilm's tumor
- don't palpate abdomen!
- manipulation of the mass may cause seeing of tumor to other sites
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late effects of child cancer treatment w/ brain radiation
slowed growth and cognitive impairment
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late effects of child cancer treatment re: musculoskeletal
- soft bone tissue and teeth
- scoliosis
- atrophy
- osteoporosis
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late effects of child cancer treatment re: reproductive
sterility
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late effects of child cancer treatment re: thyroid
hypothyroidism
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late effects of child cancer treatment re: skin
radiation permanent hyperpigmentation
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late effects of child cancer treatment re: lungs
pulmonary fibrosis
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late effects of child cancer treatment re: heart
cardiomyopathy
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late effects of child cancer treatment re: risk of second malignancies (are they common and what types to watch for)
- COMMON--
- leukemia
- soft tissue sarcomas
- thyroid cancer
- brain cancers
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the pediatric oncology nurse must be/do what
- physical presence
- communicative and understanding
- empathetic with child, parents, siblings
- become intuitive w/ experience and reflection
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