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30 Cards in this Set
- Front
- Back
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What is a port wine stain? |
this is a vascular malfromation present at birth
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does a port wine stain go away on its own?
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no it does not
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what syndrome is associated with port wine stains? risk factors?
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Sturge weber syndrome.
a intertrigeminal distribution of port wine stain. this puts them at risk for dilated capillaries in the eye, risk for gluacoma and brain vascular anomalies |
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What is a lymphangioma?
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this is a tumor of the lymph vessels characterized by cystic or cavernous spaces
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What is a cystic hygroma?
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this is found in the deep neck, axilla, mediastinum, or retropreitoneum. it is a subset of lymphangiomas
This is cystic- lymphatic space- |
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What is the most common teratoma?
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sarcoccygeal terotromas
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What are benign teratomas called?
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mature teratomas (if they are present at birth they are almost always benign)
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What are malginant teratomas called?
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immature teratoma
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what makes up a teratoma?
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all 3 germ cell layers
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What kind of tumors do you NOT get in kids?
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carincomas
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What do fetal and neonatal tumors tend to do? |
they tend to spontaneously regress- and cytodifferentiae
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are pediatric tumors anaplastic ?
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NO they are typical - Blastomas
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What does Small round blue cell tumor usually indicated?
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Wilms tumor, neuroblastoma, and lymphoma
**Characteristic histology of the most prim |
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what is the most common site of neuroblastomas?
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adrenal medulla
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What do Homer Wright pseudorosettes mean?
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these a poorly differentiated tumor cells- poor prognosis.
NEUROBLASTOMA Small round blue cell tumor- |
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are ganglioneuromas benign?
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yes they are- this is the benign version of neruoblastoma
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what are ganglioneuroblastomas?
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this is a differentiation level of neuroblastoma, between true neuroblastoma (rosettes) and ganglionueroma (benign)
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What are the clinical presentations of Neuroblastoma?
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abdominal mass
fever, weight loss can be metastatic- bone, orbit- proptosis of eye, and a Blueberry muffin belly |
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What are the two prognostic factors for kids with neuroblastoma
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Stage and Age
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What is the age range for excellent prognosis with neuroblastoma?
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less than 18 months old
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what is the age range for very poor survival of neuroblastoma?
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age 5+
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How does Ploidy related to prognosis in neuroblastoma?
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diploid is BAD- hyperploidy is good
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What gene amplification is associated with a very poor outcome in neuroblastoma?
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N-MYC
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What Trk factor is associated with a good prognosis?
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TRK-A is a good factor
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What is the most common renal tumor of childhood? benign or malignant?
TESTED! |
WILMS TUMOR- this arises from nephorgenic rests
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What does Wimls tumor come from?
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this comes from nephrogenic rests - things that didnt fully differentiate into kidney
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What two genes are associated with wilms tumor?
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WT1, and WT2 (wilms tumor)
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What is the WT-1 gene related conditions?
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Wilms tumor,
No irises gential malformation and mental retardation |
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What is the gene defect in WT-1?
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this is a chromosome 11p deletion
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What is different about Denys-Drash vs. normal WT-1?
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this is male pesudohermaphroditism
Neuropathy Renal failure (but have irises) |
