tumor Flashcards
epiphyseal tumor in a child
chondroblastoma
epiphyseal tumor in an adult
giant cell or clear cell chondrosarcoma
tumor in tibial shaft in adult and child
adult - adamantimoma, child - osteofibrous dysplasia
where will the test usually show osteosarcoma and ewing’s sarcoma in a kid?
osteosarc usually metaphyseal, ewing’s usually diaphyseal
treatment for parosteal osteosarcoma
surgery only
3 common chemos used in osteosarcoma
doxorubicin, cisplatin, high dose methotrexate
most common side effect of doxorubicin
cardiomyopathy
most common side effect of cisplatin
ototoxicity
first and second most common pediatric bone cancer
osteosarcoma first, ewings sarcoma second
positive stains in Ewings
CD99, FLI1
what stains CD1a+?
LCH
what stains CD20+?
lymphoma
what stains myogenin+?
rhabdomyosarcoma
t(11;22) EWS-FLI1
Ewing’s
chemo regimen for Ewing’s
VAC-IE - vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide
what causes fibrous dysplasia?
GNAS mutation -> g-protein defect -> increased cAMP
CD34+
angiosarcoma
what are the neoplastic cells in giant cell tumor?
preosteoblasts that express RANKL
brachyury+
chordoma
most common primary bone tumor in hand
enchondroma
soft tissue tumor deep to serratus anterior
elastofibroma, observe but remove if symptomatic
soft tissue sarcoma thats beta catenin positive
desmoid tumor
soft tissue sarcomas that go to lymph nodes
RACES - rhabdomyosarcoma, angiosarcoma, clear cell, epithelioid, synovial
translocation in myxoid liposarcoma
t(12;16), TLS-CHOP
translocation in synovial sarcoma
t(x;18) SYT/SSX
soft tissue sarcoma EMA+ and keratin+
synovial sarcoma
most common soft tissue sarcoma in children
rhabdomyosarcoma
translocation in rhabdomyosarcoma
t(2;13) PAX3/PAX7-FKHR
translocation in clear cell sarcoma
t(12;22), EWS-ATF1
soft tissue sarcoma radiation protocol
5000 cGy over 5 weeks preop, 6600 cGy over 6 weeks post op
how does photon radiation work?
creates free radicals capable of causing lethal DNA strand breaks
what does IDH1 and 2 treat?
chondrosarcoma
what is Jaffe-campanacci syndrome?
multiple NOFs, cafe au lait spots, hypogonadism, cognitive delay
what is mazabraud syndrome?
polyostotic fibrous dysplasia and myxomas
what is hand-schuller-christian syndrome?
multifocal skeletal LCH + diabetes insipidus
what is letterer-siwe syndrome?
multifocal LCH (+extra-skeletal)
CD138+
multiple myeloma
CD45+
lymphoma, leukemia
CD99+
Ewing’s
symptoms of multiple myeloma
CRAB - hyperCalcemia, renal insufficiency, anemia, bone manifestations. also prone to VTE
diaphyseal tumors
AEIOUY - adamantimoma, eosinophilic granuloma, infection, osteoid osteoma/otseoblastoma, ewing, mYeloma, lYmphoma, fibrous dYsplasia
translocation in myxoid chondrosarcoma
t(9;22), EWS-CHN
genetic association in Olliers and Mafuccis
PTHR1
summarize the Enneking staging system
I - low grade, II - high grade, III - any mets. A - intra-compartmental, B - extra-compartmental
risks of pre vs post op radiation
pre op - wound healing, post op - fibrosis
most common soft tissue sarcoma in hand and wrist
epithelioid sarcoma
most common soft tissue sarcoma in foot and ankle
synovial sarcoma
which soft tissue sarcoma requires CT abd/pelvis in addition to CT chest?
liposarcoma
inheritance of NF
autosomal dominant
rate of chondrosarcoma with regular osteochondroma and in MHE
1%, 10% in MHE
blue cell tumors
LERNM (learn ‘em) - lymphoma, ewing sarcoma, rhabdomyosarcoma, neuroblastoma, myeloma
lymphoma treatment
multiagent chemo (CHOP), consolidative radiation
treatment for adamantimoma
wide-margin surgical resection
ABC initial treatment
open curettage
UBC initial treatment
aspiration/injection
which tibial lesion is fibroosseous with rimming osteoblasts
osteofibrous dysplasia
what factors are released during bone resorption that stimulate tumor cells to multiply and release more PTHrP
TGF-B, IGF-1, calcium
loss of INI1 expression
epithelioid sarcoma (most common sarcoma of hand)
molecular pathway targeted in tenosynovial giant cell tumor of tendon sheath
CSF-1, plexidartinib
what other condition is MHE associated with?
madelung’s
chondrosarcoma treatment
wide resection
how do tumor cells metastasize
secrete PTHrP -> release of RANKL from osteoblasts -> binds RANK-R on osteoclast precursors. if G-CSF present osteoclasts activate and resorb bone, resorption releases TGF-B, IGF-1, calcium, these stimulate tumor cells to multiply and release more PTHrP
SATB2 positive
osteosarc
3 most common primary bone sarcomas
1 - osteosarc. 2 - chondrosarc. 3 - ewings
histo showing chicken wire calcifications and cobblestone appearance
chondroblastoma
genetic association with maffuccis and ollier’s
PTHR1