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31 Cards in this Set
- Front
- Back
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most common cancer in children (23%)
peak age at 2-3 years incidence highest in hispanic children, and higher in whites than blacks |
acute lymphoblastic leukemia
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**risk factors for acute lymphoblastic leukemia
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prenatal exposure to x-rays (radiation)
DOWN'S SYNDROME (also associated w/ AML) |
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treatment for acute lymphoblastic leukemia
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systemic therapy w/ specific CNS preventive therapy (intrathecal chemo)
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**bone marrow characteristics to make the diagnosis for acute myeloblastic leukemia
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marrow must include greater than 20% leukemic blasts
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treatment for acute myeloid leukemia
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mainstay is systemically administered combination chemo
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leukemia characterized by the presence of the Philadelphia chromosome
most patients are 6 years or older |
chronic myelocytic leukemia
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leukemia caused by malignant transformation of a primitive hematopoietic stem cell or progenitor in kids under 2yrs of age
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juvenile myelomonocytic leukemia
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disorder that can be difficult to distinguish from AML (may occur in infants w/ genetic mosaicism in the bone marrow for trisomy 21)
clonal expansion of myeloblasts most frequently megakaryoblasts usually spontaneously remitts in the first 3 months of life |
transient myeloproliferative disorder (TMD), not a malignant process
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the third most common childhood malignancy
what type is the most frequent malignancy in children w/ AIDS |
lymphoma (Hodgkin's and non-Hodgkin's)
with AIDS: NHL |
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most important prognostic determinant in the treatment of NHL
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the extent of disease at diagnosis
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tumor of early childhood that originates in the adrenal medulla or paraspinal sites
Symptoms inlude: Bone pain (from mets) proptosis (eyes sticking out) and periorbital ecchymosis from retrobulbar mets, abdominal distension and respiratory compormise (from massive liver mets), and may compress spinal nerves (since it arises in paraspinal ganglia) |
neuroblastoma - arises where the sympathetic nervous sytem tissue is present
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common symptoms of neuroblastoma
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abdominal distention w/ respiratory compromise due to huge liver
paralysis due to paraspinal invasion into cord fever, anemia, htn |
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treatment of neuroblastoma, as stratified by risk
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low risk: surgery alone, chemo for symptoms
intermediate risk: surgery and 12-24 weeks of chemo high risk: aggressive multiagent chemo followed by resection of primary, followed by myeloablative chemo and sometimes radiation or stem cell transplantation |
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bone tumor that occurs predominantly in adolescents and young adults
in the pediatric population, 80% of these originate around the knee |
osteosarcoma
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significant prognostic factor for localized osteosarcoma
what is the most significant prognostic factor for both metastatic and localized osteosarcoma? |
site of the primary tumor (axial skeleton greatest risk of progressing to death)
the most significant factor is the resectability of the tumor because very resistant to chemo. note: degree of necrosis, 95% necrosis after chemo is associated w/ a better prognosis |
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most common site for osteosarcoma metastases
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lung
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soft tissue malignant tumor of skeletal muscle origin
localized form is treatable w/ combined modality therapy |
rhabdomyosarcoma
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genetic conditions associated w/ rhabdomyosarcomas
Note: most are sporadic in origin w/ no predisposing factor |
Li-Fraumeni syndrome
Neurofibromatosis type I Beckwith-Wiedemann syndrome |
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important step in the clinical evaluation of suspected rhabdomyosarcoma
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before biopsy of a suspected tumor mass, imaging studies and labs should be obtained
check these kids over really well for primaries or mets |
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family of tumors that occurs most frequently in the second decade of life
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Ewing's Family of Tumors
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most common of the Ewing's Family of Tumors
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Ewing's sarcoma of bone (60%)
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most common location of Ewing's sarcoma of bone
most common location of extraosseous Ewing's and primitive neuroectodermal tumors |
ETB: extremeties
EOE and PNETs: trunk |
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treatment for Ewing's sarcoma
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multidrug chemo along with radiation and/or surgery
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most common soft tissue sarcomas reported in children
this is also the soft tissue sarcomas that benefit from chemo (the others are questionable, radiation and surgery more common) most commonly found in the extremities |
synovial sarcomas
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anomalies associated w/ Wilm's tumor
what is the most widely known gene involved, and what is its function? |
hemihypertrophy
cryptorchidism hypospadias the WT1 gene on chromosome 11 is frequently implicated, and it is responsible for differentiation of the renal blastema |
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2yr-old Patient presents w/ hepatosplenomegaly, lymphadenopathy, fever, rash, leukocytosis w/ INCREASED CIRCULATING MONOCYTES and elevated HEMOGLOBIN F, dx
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juvenile myelomonocytic leukemia (JMML)
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Neuroblastomas cannot be differentiated from other small round-blue cell tumors of childhood (lymphomas, rhabdomyosacromas..), so what test can help in its diagnosis.
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Immunohisotchemistry
Electron microscopy Elevated serum or urine catecholamines |
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Which patients with neuroblastoma have the worst prognosis?
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adolescent or adult has worst long-term prognosis regardless of stage or site
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How are patients w/ malignant fibrous histocytoma (MFH) treated
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same as osteosarcoma, and outcome for resectable MFH is similar as well
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Most common sites for rhabdomyosarcoma
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1. head and neck
2. genitourinary tract 3. extremities |
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what is the gene involved in Li-Fraumeni syndrome
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p53 tumor suppressor gene
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