Neoplasm Flashcards
acute lymphocytic leukemia
most common childhood ca-80%
peak 2-6yo
Labs: low rbi/plt. wbc-1/3high, 1/3 nl, 1/3 low. leukemic blasts. nl CBC doesn’t r/o ALL, elevated uric acid and LDH. confirm with BM eval- see marginalization of lymphoblast bone marrow show incr early pre-B lymphoblasts (70%, of this 70% CALLA pos), 25% T cell phenotype
P: fever, bone or J pain- can’t bear wt = limp, pallor, bruising, hsm, lymphadenopathy,
petechiae + URI
think systemic infection like meningococcemia, sepsis, vascullitc process, coagluopathies, things that interfere with BM ability to make plot ~ malig, infiltrative process or aplastic crises.
cancer
leading cause of death from disease in kids: leukemia, brain tumor, lymphoma, neuroblastoma
RF: genetics, immunodef, infectious disease (EBV and HIVwith burkitt’s lymphoma and KS respectively
P: fever, abd mass is malig until proven otherwise (wilm’s or neuroblastoma), bone pain, suprclavicular lymphadenopathy (nontender, firm), early morning HA/V, bruising, petechiae pallor, leukocoria (white reflex in pupil, HTN (Wilm’s, phew, neuroblastoma)
acute lymphoma prog
good: 1-9yo, female, white, WBC>50K, hyperploidy, no organ involved, CALLA pos, no chromosome translocation
ALL tx
3 stages
1) induction: destroy ca cell= induce remission. give intrathecal methotrexate to all. add steroids, vincristine etc
2) cosolidation: cont systemic chemo and prophylactic regimens to prevent CNS. cotin intrathecal methotrexate, crandial irradiation for high risk
3) maintenance: daily periodic chemo during remission for up to 3y
BM transplant for replase or very sick
good prog- 85% long term survival
tumor lysis syndrome
therapy or spontaneous cell lysis causing metabolic problem
hyperuricemia result in renal infuse
hyperkalemia in cardiac dyshyrthmia
hyperphosphatemia- hypercalemia with tetany
acute myelogenous leukemia (AML)
associated with Down’s. more CNS involvement than in ALL
P: fever, hsm, bruising, givgival hypertrophy, bone pain,
labs: pancytopenia or leukocytosis and DIC
dx: blood smear with leukemic myeloblast. blasts with Bauer rods. confirm with BM bx.
tx: myeloablative therapy. BM transplant rec once in remission
prog: 50% recover with chemo. 70% w/ BM transplant.
chronic myelogenous leukemia (CML)
least common type of leukemia.
1) adult type: philadelphia chromosome: translocation btw 9/22- BCR-ACL. P-nonspecific sx like fatigue, wt loss, pain, Massive splenomegaly- abd distention, extremely high WBC> 100K
2) juvenile chronic myelogenous leukemia: in
hodgkin’s lymphoma
ca of ag processing cells in LN or spleen. associate with EBV infection
P: painless lymphadenopathy- supraclavicular/cervical, slow onset, common systemic sx, rare abd finding, no SVC syndrome/airway compression
dx: LN bx- reed sternberg cell
tx: rad + chemo. C of tx_ growth retardation due to rad, secondary malig, hypothyroidism, male sterility
good prog for stage 1/2
non hodgkin lymphoma
more common and aggressive than hodgkin’s
male predom, immunodef sates-HIV, wiskott-aldrich, ataxia telangiectasia and EBV
P: rapid onset, abd, mediastinal and supraclavicular nodes, no systmeic sx, commonly has abd findings, painless adenopathy, SVC syndrome, airway compression
types:
1) lymphoblastic lymphoma- T cell: anterior mediastinal mass, SVC syndrome - airway obstruction
2) burkitt’s -B cell. inttussuception (any kid >3 need r/o lymphoma), jaw mass
3)large cell lymphoma - b cell. tonsil/peyer’s patch enlarge
dx: CT, BM bx, CSF analysis
tx: debulk, chemo, prophyylazis for CNS
prog: 90% for localized. bad for disseminated
brain tumor
most common solid tumor
associated with NF, TS, vHL
in order from most common: glial cell most common, then PNET (~ medulloblastoma), ependymomas, craniopharyngioma
brain tumor sx
initially due to incr ICP. worse in sleep and on awakening. improve during day as venous return improve with upright posture
P: head tilt, optic glioma- diminished vision,strabismus. craniopharyngioma- growth retardation, delay puberty
tx: debulk, rad if >5yo. chemo.
neuroblastoma
malig tumor of neural crest cell that may arise anywhere along sympathetic ganglia chain and in adrenal medulla
2nd most common solid tumor.
peak in 1st 5y of life. 75% in abd or pelvis
genetics: deletion in C1, tr1p7q, or C14,22
Dx: excessive catecholamine -VMA, HVA. BM bx. CT to assess spread, skeletal survey to assess meta
neuroblastoma sx
abd location- firm abd mass that cross midline. abd pain, anorexia.
if mediastinal- respiratory distress
catecholamine effects
nonspecific- fever wt loss
metastatic disease in 70% at dc- periorbital exxhymosiss, proptosis, skin nodule with blueberry muffin appearance, bone pain, limp, hepatomegaly
neublastoma tx
surgery curative for stage 1/2. chemo+rad for later. poor prog with III, IV, amp of N-myc, high ferritin , LDH, neuron specific annuals
