Heme/Onc Flashcards
What is PHACE(S) syndrome?
posterior fossa malformations large facial Hemangiomas Arterial abnormalities Coarctation of aorta Eye abnormalities Sternal defects
–> have unilateral facial lesion and blindness
What is glomus tumor triad?
severe intermittent pain, tenderness, sensistive to touch
= benign vascular tumor often subungual
When should you suspect infantile fibrosarcoma?
hemangioma that is not resolving as expected
What is pattern of osteosarcoma? where in bone?
MC primary bone tumor in kids, in metaphysis
local pain/swelling, no constitutional sx, sunburst pattern, periosteal elevation
high alk phos/LDH
What is pattern of osteiod osteoma?
pain at night that responds to NSAIDS
What is pattern of langerhans cell histiocytosis?
solitary lytic bone lesion, swelling, high Ca
What is pattern of ewing sarcoma?
metaphysis/diaphysis of femur, pain/swelling for wks-mos, red/warm
Dx: onion skin periosteal reaction, lytic, endosteal scalloping
early met to lungs and lymph nodes
What is first dx step if suspect hodgkin?
CXR to look for mediastinal mass
How do you dx ALL?
more than 25% lymphoblasts on BM
What are sx of ALL?
thrombocytopenia, bone pain, HSM, anemia, lymphadenopathy
How do granulosa cel tumors present pre-puberty?
precocious puberty due to excess estrogen
What is WAGR?
wilms, aniridia, GU, mental retardation, hemihypertrophy
What is beckwith wiedmann?
macrosomia w/ macroglossia, ab wall defects, organomegaly, often hypoglycemia in newborn
have wilms, hepatoblastoma, gonadoblastoma
How do you dx wilms?
ab US
How do you dx neuroblastoma?
high HVA/MVA
