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18 Cards in this Set
- Front
- Back
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Vascular tumors are predominately?
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arterial. rapid neonatal growth.
malformations are usually venous with no change in size. |
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Kasabach Merrit associatios?
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Tufted hemangioma, Kaposiform Hmenagioendothelioma.
Tx-prednisone, vincristine, IFN-a |
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PHACE syndrome?
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X-linked dominant.
Posteriof fossa malformations Hemangioma (large, facial) Arterial abnormalities (coarctation) Cardiac (VSD, PDA) Eye abnormalities Sternal clefting/supraumbilical raphe |
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Sturge Weber (Encepholotrigeminal angiomatosis)
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Sporadic,
10 % with V1 distribution, usually unilateral. hypertrophy of underlying structures. CNS-seizures, MR, tramtrak calcifications in temporal and occipital cortex. Can have ipsilateral glaucoma. |
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Cobb syndrome (cutaneomeningeal angiomatosis)
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AV of venous angioma of spinal cord associated with dermal distribution on back.
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HHT
(Osler-Weber-Rendu) |
AD
HHT1 (endoglin gene-assoc. with pulm AVM's), HHT2 (ALK1 gene) (assoc. with hepatic AVM's) -epistaxis in 50% -telangiectasisas on face, palms, soles -Pulm AVM's -GI telangiectasia with secondary hemorrhage |
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Fabry Syndrome
(angiokeratoma corporis diffusum) |
XLR.
Defect in alpha galactosidase A (lysosomal storage disease) -Angiokeratomas in bathing suit distribution -Periph paresthesias, htn, hypohidrosis -Maltese cross on UA |
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Ataxia Telangiectasia (Louis Barr Syndrome)
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AR
Defect in ATM gene-DNA repair. Telangiectasisas on conjunctiva, face and ears. Cerebellar ataxia, progressive nystagmus -increased risk of lymphoma, leukemia, breast CA. |
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von Hippel-Lindau
(Angiomatosis retinae et cerebelli) |
AD,
VHL gene (tumor suppressor) retinal hemangiomblastomas RCC Pheochromocytoma, pancreatic cysts, adrenal ca Universally fatal by 4th decade |
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Klippel-Trenaunay
(Angio-osteohypertrophy syndrome) |
Sporadic
Angiogenic factor v65, RASA1 cap malformation LE, venous varicosity, hyperlasia ST and dome -If AV fistula is present it is called Parkes-Webber |
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Pregnancy epulis
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Pyogenic granuloma in pregnancy
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5 types of Kaposi's Sarcoma
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1. Classic-legs, feet, nodular
2. African cutaneous, < 10yo and > 20 yo 3. African lemphadenopathic < 10yo 4. AIDS, head and neck 5. Immunosuppresive (about 30 mos. after transplantation) |
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Proteus syndrome
(Riley-Smith and Banyaan) |
PTEN gene-mosaic mutation
-cerebriform feet, capillary malformations, macrocepharly, asymmetrc st or bone hypertrophy |
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Beckwith-Wiedeman Syndrome
(Exopthalmos-macroglossia-gigantism or EMG syndrome) |
p57 KIP gene
-TONGUE Tumors Omphalocoele Neonatal hypoglycemia Gigantism U/S Ear lob creases |
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Cutis Marmorata Telangiectasia Congenita
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Sporadic.
MSK-limb length discrepancy CV-PDA, arterial stenosis Eyes-glaucoma CNS-MR |
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Maffucci syndrome
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Superficial and deep venous malformations (PTH1 receptor)
Short stature Endochrondromas Chrondrosarcomas (15-20%) |
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Blue Rubber Nevus Syndrome
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TIE2 gene, AD
multiple venous malformations GI-venous malformations with secondary hemorrhage and anemia |
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Diffuse Neonatal Hemangiomatosis
(Mult neonatal hemangiomatosis) |
Liver hemangiomas
High output CHF |
