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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
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what are Chromosomal fragility syndromes ~ leukemia?
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Down’s , Blooms syndrome,-(chromosomal breakage d/o, immunodef, short, infertile, facial anomalies) Fanconi’s anemia
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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) |
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onion skin pattern on Xray; what chromosomal translocation associated w/ ?
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~ t (11:22), WES-FLI;
Ewings, radiation sensitive |
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• 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
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where is mets most common? in bone tumor of sunburst pattern, Codman's triangle?
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Osteogenic sarcoma;
mets to lungs; also associated w/ radiation to retinoblastoma |
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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
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tibia or femur pain, worse @ night, relieved by ibuprofen
-Xray: central radiolucent (white are) surrounded w/ thick sclerotic bone |
Osteoid Osteoma
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-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
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DX: + Reed Sternberg, hi WBC, low lymphocyte? what disease; ~ with what syndromes
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Hodgkin's lymphoma
~ ataxia teleng; AIDS; AML- most common secondary malignancy |
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-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)
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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)
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? Poor progostic factors for ALL: 4
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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) |
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good Prognostic factors for ALL
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good Prognosis: Pre-B cell; WBC < 10 k; CALLA +
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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
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Hi risk and good prognosis for AML?
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High risk: monosomy 7, 5, 5q;
Good prog: Down’s |
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AML ~ what syndromes
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~ Klinefelters, Blooms, Fanconi’s NF, Kostman’s, Schwachman Diamond; Hodgkins
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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
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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
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bone lesions, exophtlamos, DI)
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Hand Schuller Christian diz
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what are 3 good prognostic factors for LCH?
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Good: localized, older child, eosinohpilic granulmoa;
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3 bad prognostic factors for LCH?
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Poor: < 2yo; preschool, handshculler Christian,; worse for infant
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-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)
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? how diagnose nueroblastoma?
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DX: tumor bx; hi VMA, HMA; BM findings; pancytopenia, hi ferritin, hi LDH
-blasts on peripheral smear |
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GOod prognostic factors for neuroblastoma?
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Prognosis: good: < 18 mo, n myc- non amplified; hyperdiploid; localized diz (neg bone scan)
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Bad prognostic factors for neuroblastoma?
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Poor: nmyc aplification; 1 p- chromosola dletion; Hi ferritin ; diploid or hyperdiploid
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** 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
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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 |
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How diagnose Wilms?
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DX: CT: distorted renal parenchyma vs neuroblastoma;
No calcifications; |
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3 good prognostic factors for wilms?
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Good: age < 2yo, histology – more impt than stage, , age (>2 worse)
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3 bad prognostic factors for wilms?
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**Unfavorable: anaplastic, clear cell (mets to BONE & brain); > 2 yo
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what other syndromes is Wilms associated with ?
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~ 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” |
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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
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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 |
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What's genetics and chromosomal loss of retinoblastoma?
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B/L diz: AD (? Nabil baky)~ 13 q: loss of Rb tumor suppressor gene
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most common secondary malignancy ~ Retinoblastoma?
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~ Osteogenic sarcoma –
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