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36 Cards in this Set

  • Front
  • Back
recurrent epistaxis, mucous membrane/lip lesions around puberty
-cuaneous lesions-face, pinna, palms, nail beds
-fistulous vascular communications in lungs→decreased exercise tolerance
-clubbing, polycythemia
Osler Weber Rendu
what are Chromosomal fragility syndromes ~ leukemia?
Down’s , Blooms syndrome,-(chromosomal breakage d/o, immunodef, short, infertile, facial anomalies) Fanconi’s anemia
Painful bony lesion, Soft tissue component
~NOT seen in African Americans and Asians
-mid teen years
-Most common in midshaft (middle) of long bones, flat bones (not metaphyseal like osteogenic sarcoma
~ F, wt loss, fatigue
• Ewings
** painful vs OS; not seen in Blacks/asians (vs OS - seen in blacks)
onion skin pattern on Xray; what chromosomal translocation associated w/ ?
~ t (11:22), WES-FLI;
Ewings, radiation sensitive
• more common- Blacks > whites
-Metpahyses (neck) of long bones: proximal tibia/distal femur)
-painless
teenager going thru growth spurt (10-20 yo ) (U/L vs growing pains: B/L !!)
Osteogenic Sarcoma
where is mets most common? in bone tumor of sunburst pattern, Codman's triangle?
Osteogenic sarcoma;
mets to lungs; also associated w/ radiation to retinoblastoma
Both – involve bones, Long bones
- more common in second decade of life
-TX: chemo, radiation, surgery
-Both: metastatize to lungs but this is main site of OS mets
both: ewings, osteogenic sarcoma
tibia or femur pain, worse @ night, relieved by ibuprofen
-Xray: central radiolucent (white are) surrounded w/ thick sclerotic bone
Osteoid Osteoma
-teens, mid-late 20s, then > 60
nontender enlarged cervical lymph nodes; wt loss, F, night sweats
-slow growing; cervical nodes- 1st, then medastinal, abdominal, hen pelvis ;painless supraclavic or clavicular LAD
Sx: nontender cervical node or supraclavicular node;
Hodgkin’s Lymphoma
DX: + Reed Sternberg, hi WBC, low lymphocyte? what disease; ~ with what syndromes
Hodgkin's lymphoma
~ ataxia teleng; AIDS; AML- most common secondary malignancy
-small child
- involves head/neck region; also abdomen as non-tender mass
- Lymphobalstic: T cell; mediastinal masses, pleural effusions
-Non-lymphoblastic: B cell
Dx: < 25% blasts (vs leukemia : > 25 % leukemia)
Dx: < 25% blasts (vs leukemia : > 25 % leukemia)
most common peds malignancy
-Pk : 4 yrs
SX: “bone pain” and/or “joint pain or swelling”, + Fevers, bruising, LAD, pancytopenia, tho WBC ~ normal or hi;
+ HSPM (vs aplastic anemia),
Dx: bone marrow > 25% blasts
ALL (acute lymphoblastic leukemia)
? Poor progostic factors for ALL: 4
1) Younger than 2, > 10 yo @ dx
2) WBC > 50
3) T cell- Terrible; mature B cell (burkitt’s – worse) ; testicular diz
4) t (9:22)
good Prognostic factors for ALL
good Prognosis: Pre-B cell; WBC < 10 k; CALLA +
older kids
-worse prognosis
- Bone marrow: varied pic
-~ presentation of ALL; except never involves testicles

-no peak age or sex
- leukjoerythroblastic rxn
-primitive WBC, nucleated RBC , tear drop)
DX: Aurer rods
AML (m1, M2) – acute myelgenous leukemia: nonlymphobastic leukemia
Hi risk and good prognosis for AML?
High risk: monosomy 7, 5, 5q;
Good prog: Down’s
AML ~ what syndromes
~ Klinefelters, Blooms, Fanconi’s NF, Kostman’s, Schwachman Diamond; Hodgkins
Mean age: mid 40s
-persistent neutrophila w/o infections; + basophilia
-most common cz of splenomegaly; ~ F, cervical LAD,
+ t (9:22): Philadelphia chrom
- “starry sky”
CML
Sx: seborrheic rash, Ear d/c; Lytic lesions in skull
~ DI, excessive urination
Dx: skin bx and EM: “birbeck granules
Langerhans Cell Histiocytosis ( long Hands Hysterical X) – not malignancy
bone lesions, exophtlamos, DI)
Hand Schuller Christian diz
what are 3 good prognostic factors for LCH?
Good: localized, older child, eosinohpilic granulmoa;
3 bad prognostic factors for LCH?
Poor: < 2yo; preschool, handshculler Christian,; worse for infant
-non tender abd mass; most common solid tumor in chilhood; wt loss, anorexia; pallor; < 4 yo; UTI from obstructing abd mass
-UTI, Raccoon eyes:, proptosis leg/hip pain
-Horner syndrome (due to mediatstinal tumor compressing recurrent larygneal nerve
-irrtibality, HTN diarrhea~ hi VIP; -opsoclonus/myoclonus
~ skin mets: blueberry muffin rash
Neuroblastoma (adrenal gland)
? how diagnose nueroblastoma?
DX: tumor bx; hi VMA, HMA; BM findings; pancytopenia, hi ferritin, hi LDH
-blasts on peripheral smear
GOod prognostic factors for neuroblastoma?
Prognosis: good: < 18 mo, n myc- non amplified; hyperdiploid; localized diz (neg bone scan)
Bad prognostic factors for neuroblastoma?
Poor: nmyc aplification; 1 p- chromosola dletion; Hi ferritin ; diploid or hyperdiploid
** 70% metastatic at dx: liver, spleen, bone marrow, Lymph nodes; only solid tumor outside CNS that doesn’t met to lungs
~ Hirsgprung’s fetal hydantoin, von Recklinghausen’s
neuroblastoma
most common peds abd malignancy;
-~ 3.5 yo
-painless abd mass, HTN, gross hematuria
~ WAGR:
-hemihypertrophy curved tongue
Wilms;
WAGR: wilms/Weidman (beckwith);
-Aniridia
-Genital /urogenital tract probs
-Retardation
How diagnose Wilms?
DX: CT: distorted renal parenchyma vs neuroblastoma;
No calcifications;
3 good prognostic factors for wilms?
Good: age < 2yo, histology – more impt than stage, , age (>2 worse)
3 bad prognostic factors for wilms?
**Unfavorable: anaplastic, clear cell (mets to BONE & brain); > 2 yo
what other syndromes is Wilms associated with ?
~ NF, Betweith wiedeman syndrome (exophtalmos, macroglossia, gigantism, neonatal hypoglycemia, hepatoblastoma)
Mneum: “wide Man” in Fred flinstone; Wilms as Wilma: fred and Wilma flinstone sharing “half a body”
most common soft tissue sarcoma in kids
-h/o constipation; rectal exam + mass: visible or palpated;
-grape like mass protruding from vagina
- Young pts tend to have head/neck rhabdo, older (teens) – truncal or extrem tumors
Rhabdomysarcoma
Pk- 18 mo;
-early strabismus
-leukokoria (white pupil): abnl red reflex– most common, glaucoma;
late- proptosis, bone pain, increased ICP
-Photophobia, increased tearing in neonate – more~ cong glaucoma
Retinoblastoma
**Photophobia and tearing in neonate more suggestive of of congenital glaucoma
What's genetics and chromosomal loss of retinoblastoma?
B/L diz: AD (? Nabil baky)~ 13 q: loss of Rb tumor suppressor gene
most common secondary malignancy ~ Retinoblastoma?
~ Osteogenic sarcoma –