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31 Cards in this Set
- Front
- Back
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most common benign neoplasm of children
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hemangioma
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what is a strawberry hemangioma
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capillary hemangioma of infants
fades by school age |
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what isa port wine stain
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vascular malformation present at birth that does not fade with time
dilated dermal vessels of the face |
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what is Sturge-Weber synd
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plort wine stain with neurological components
trigeminal distribution risk of glaucoma |
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what is a lymphangioma
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benign tumor of lymph vessels that can grow large enough to impinge on other structures
cystic or cavernous spaces neoplasm or hamartoma |
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where do you find lymphangiomas
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skin
cystic hygroma; neck, axilla, mediastinum, retroperitoneum |
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what are the two fibrous tumors mentioned
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fibramatoses- benign
congenital infantile fibrosarcoma- sarcoma of infants with a good prognosis |
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what is a childhood teratoma
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present at birth, slow growing , pearks at 2yrs
most commonly in the sacrococcygeal region (also, head, neck, mediastinum) arises from totipotent germ cell rests |
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what is the nomenclature for a benign and malignant teratoma
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benign- mature
malignant- immature |
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histological differences in childhood tumors
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blastoma: embryonal appearance with features of organogenesis
anaplasia less common |
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what is the epidemiology of a neuroblastoma
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2nd most common solid tumor of children
most <5yrs survival rate 40% |
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where do you find neuroblastomas in children
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Adrenal medulla- most common
paravertebral posterior mediastinum |
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microscopy of a neuroblastoma
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SRBCT
homer wright pseudorosetts- blue cells around a fluid core |
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what are the two more mature forms of neuroblastoma
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ganglioneuroblastoma- various stages of differentiation
ganglioneuroma- mostly mature ganglion cells & schwann cells |
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what are the stages of neuroblastomas
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stage I- localized, no metastases
Stage III- extends across the midline, unresectable Stage IV- has metastasized to bone & liver Stage IVs- extensive metastases to skin, liver, and bone marrow |
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clinical manifestations of neuroblastoma
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abdominal mass
fever, weight loss elevated Catecholamines, VMA, VA, HVA poss metastatic disease blueberry muffin nerve impingement |
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prognosis of neuroblastoma
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excellent- stage I and II, <18 mo. at any stage
intermediate- 18 mon to 5 yrs extremely poor- >5yrs |
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prognostic factors for neuroblastoma
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ploidy: hyperploidy good, diploidy bad
N-myc- homogenous staining region and double minutes Trk-A: nerve growth factor receptor, Good prognosis Trk-B: Poor prognosis, treated with TKI's 17q+, 1p-, 11q- |
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what is the other name for Wilms tumor
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nephroblastoma
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what are the 3 genetic predispositions for wilms tumor
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WAGR- WT-1 mutation
Denys-Drash- WT-1 mutation Beckwith-Wiedemann synd- WT-2 mutation |
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what is a wilms tumor
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renal tumor of childhood
peak= 2-5 rys |
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what is the WT-1 gene
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tumor suppressor gene on chromosome 11
transcription factor for normal renal and gonadal development |
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what is WAGR
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Wilms, aniridia, genital malformation, retardation
Chromo 11p deletion |
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what is Denys-Drash
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male pseudohermaphroditism& nephropathy
missense mutation of WT-1 |
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what is beckwith-weidermann synd
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enlarged organs
hemihypertrophy adrenal cortical cytomegaly insulin like GF 2 overexpression |
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morphology of wilms tumor
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nephrogenic rests
triphasic microscopy-blastema, stroma, epithelial cell types |
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clinical presentation of nephroblastoma
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abdominal mass c pain
hematuria hypertension (obstruction) good prognosis |
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gene involved in RB
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RB1
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microscopic presentation of RB
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neuroepithelial cells of the posterior retina
SRBC for a flexor-wintersteiner rosette |
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clinical presentation of RB
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around 2 yrs
cats eye reflex- leukocoria poor vision and strabismus |
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treatment of RB
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enucleation followed by radiation therapy and chemo
good prognosis |
