oncoped Flashcards
most common childhood malignancy; increased incidence in Down syndrome; ataxia-telangiectasia; Fanconi syndrome; NF1; risk is >70% if the first twin is diagnosed during the 1st yr of life and they shared the same placenta; patients who have undergone intense Tx for a solid tumor
ALL
Diagnoses by a bone marrow evaluation that shows >25% of the BM cells as a homogenous population of lymphoblasts
ALL
most important prognostic factor in ALL
treatment
3 of the most important predictive factors in ALL
age at the time of diagnosis; intitial leukocyte count; speed of response to treatment
Treatment of ALL Remission induction
Vincristine; prednisone; L-asparaginase x4wks + CNS prophylactic irradiation or chemotherapy to eradicate leukemic cells from BM
Tx of ALL Maintenance phase
Mercaptopurine daily and Methothrexate weekly x2-3yrs
complication of therapy in ALL
tumor lysis syndrome
definition of remission in ALL
<5% blasts in the BM and a return of neutrophil and platelet count to near-normal levels after 4.5wks of tx
site of metastasis of ALL
liver; spleen; lymph nodes
poor prognostic factors in ALL
10 yrs; male; WBC>100k U/L on presentation; presence of CNS leukemia; presence of mediastinal mass
Ann Arbor staging of lymphomas
Stage 1-one LN group involved; Stage II-two LN groups involved on the same side of the diaphragm; Stage III-two LN groups involved on both sides of the diaphragm or extralymphatic involvement; Stage IV-diffuse extralymphatic site involvement (usually BM CNS or liver)
B symptoms in lymphoma
weight loss >10% of TBW over 3mos; unexplained high-grade fever; drenching night sweats)
Chemotherapeutic agenets for Hodgkin’s lymphoma
COPAD Cyclophosphamide; Vincristine; prednisine; Doxorubicin) or COMP (Cylophosphamide; vincristine; methotrexate; prednisone; 6mercaptopurine)
treatment of hodgkin’s in patients with refractory disease or relapse
myeloblative autologous stem cell transplant
3rd most common posterior fossa tumor of childhood infiltrates the pons and spreads throughout the brainstem
brain stem glioma
common supratentorial tumor in children with solid and cystic areas that tend to calcify; shor stature; hypothyroidism; DI; adrenal insufficiency
craniopharyngioma
most common malignant abdominal tumor
Wilm’s tumor?
Wilm’s tumor is associated with what dse entities
neurofibromatosis; Beckwith-Wiedemann syndrome; WAGR syndrome
hemihypertophy; visceromegaly; macroglossia
Becwith-Weidememann syndrome
WAGR syndrome
Wilm’s tumor; aniridia; genito-urinary abnormalities; mental Retardation
painless abdominal mass with flank mass that DOES NOT cross the midline
Wilm’s tumor
3rd most common pediatric cancer
neuroblastoma
pluripotent stem cells invaginate and migrate along the neuraxis where neural crest cells are present (sympathetic ganglia; adrenal glands)
neuroblastoma
most common extracranial tumors in infants
neuroblastoma
nontender abdominal mass which may cross the midline; Horner syndrome; HTN; cord compression(from a paraspinal tumor)
neuroblastoma
most common sites of metastasis in neuroblastoma
long bones; skull; bone marow; liver; lymph nodes; skin
Tx for St1(confined to structure of orgin) and St2(tumor beyond structure of origin but not cross the midline with 2B or without 2A LN involvement
surgery
a tumor of striated muscle; most common soft tissue sarcoma in children
rhabdomyosarcoma
histologic type of rhabdomyosarcoma with the worst prognosis
alveolar
most common histologic type of rhabdomyosarcoma
embryonal
vaginal grape-like mass type of rhabdomyosarcoma
sarcoma botyroides
highest risk period for developing osteosarcoma
adolescent growth spurt
risk factor in developing osteosarcoma
hereditary retinoblastoma
most common SSx in osteosarcoma
pain; limp; swelling
px with deep bone pain often nighttime awakening; palpable mass; xray demonstrating the lesion
osteosarcoma
Xray finding in osteosarcoma
sunburst pattern (due to mineralization and formation of periosteal spicules in the surrounding tissues
small; round cell undifferentiated tumor with small uniform nuclei and scant cytoplasm thought to be of neural crest origin; with systemic manifestations like fever and weight loss unlike osteosarcoma
Ewing’s sarcoma
most likely chromosomal aberration in Ewing’s sarcoma
t(11;22) translocation
Xray finding in Ewing’s sarcoma
onion-skinning or moth-eaten appearance (lytic bone lesion with periosteal reaction)
what part of the bone is the most common location of osteosarcoma
metaphysis
what part of the bone is the most common location of Ewing’s sarcoma
diaphysis
