Ginger Holt Video Flashcards
Sarcomas are tumors of?
connective tissue
sarcomas typically mets vis
hematogenous spread and not nodal spread, mets to chest and lungs
age less than 5 benign
Osteomyelitis
Osteofibrous Dysplasia
age < 5 malignant
LCH (Letter-Siwe)
LCH (hand schuller)
Metastatic Neuroblastoma
age < 30 malignant
ewings
osteosarcoma
age < 30 benign
Osteoid Osteoma Osteoblastoma Chrondroblastoma ABC LCH Osteofibrous Displasia Non-Ossifying Fibroma
> 30 malignant
Chondrosarcoma Mets Lymphoma Myeloma Chordoma Adamantinoma
Age > 30 benign
GCT
Paget’s DZ
DX? Diurnal pain, relief with NSAIDS (aspirin non selective)
Osteoid Osteoma
*soft tissue sarcomas are deceptively painless
11,22
Ewings
X-18
Synovial Sarcoma
SSX1-SYT
Synovial Sarcoma
RB and p53
Osteosarcoma
EXR/CRP
Ewings and Infection
SPEP/UPEP
Myeloma
PSA
Prostate
p53 is on chromosome ?
17
retinoblastoma gene is on chromosome
13
Limb salavage surgery/Wide excision
(not necessarily amputation is tx for what?
Chondrosarcoma Adamantinoma Chordoma PARosteal Osteosarcoma Soft tissue sarcoma
ORIF + radiation for which processes
Mets (Prostate, Breast, Lung Kidney, Thyroid)
Lymphoma
Myeloma
RFA
Osteoid Osteoma
Intra-lesional resection is ok for which tumors…
GCT
ABC
NOF
LCH
Doxorubicin toxicity and mech of ACTION
Cardiac toxicity
anthracycline antibiotic
Cytostatic agent
after doxorubicin tx of bone sarcoma patient needs what study
echocardiogram
Preop radiation dose for soft tissue sarcoma
50 Gy
Postop radiation dose for soft tissue sarcoma
60-66 Gy
radiation dose for mets
25-30 Gy
*whole bone/surgical field needs radiation
Downside to preop radiation therapy
periop wound healing complications
Downside to postop radiation therapy
LARGER FIELD, long term fibrosis, and bone fractures, higher rate of radiation induced sarcomas
Heterotopic bone ppx dosage
5GY single dose, 4 hours before or within 72 hours after
ortho bullets says < 550cGY not effecting and says dose i 700cGy
ADVANTAGE OF preop radiation
lower dose and smaller field
remove tumor but don’t have wide cuff of tissue=
marginal resection
marginal resection is OK for which sarcoma
Liposarcoma
Schwannoma
most common type of sarcoma resection, takes normal cuff of tissue
wide resection
*limb salvage surgery
removal of entire compartment as a form of tumor resection
radical or amputation
In order most common sites for metastatic disease spread
Lung
Liver
Bone
In order most common sites of spread for bone mets
Spine
Proximal Femur- most comon site of fracture
Humerus-second most common site of fracture
most common carcinomas that metastasize to bone
BLT with a Kosher Pickle breast lung thyroid kidney prostate
order of tumor workup
History Local staging- Xray, CT scan MRI, systemic staging biopsy
ap xray of every bone in the body
Skeletal Survey
next step in DIAGNOSIS of myeloma
SPEP/UPEP
next step in STAGING of myeloma
Skeletal Survey
next step in staging of presumed metastatic lytic lesion of bone
CT of Chest, Abdomen and Pelvis
for OSA, CSA, EWS, STST you need ct of what only
CT chest, not CAP
whole body bone scan needed for staging in which cases
OSA
EWS
METS
Bone marrow biopsy needed for staging in what cases
mYeloma with mets, and EWINGS
Smooth muscle actin +
Leiomyosarcoma
Desmin + (skeletal muscle)
Rhabdomyosarcoma
S-100 + (neural)
Schwannoma, MPNST
CD34/CD31 + (endothelial cells, vascular tumors)
Hemangioma
Angiosarcoma
Beta Catenin+ (Wnt signaling)
fibromatosis, extra-abdominal desmoid
CD99+
ewings/PNET
IHC: Keratin+
Epithelial sarcoma
Synovial Sarcoma
Carcinoma
IHC: EMA (epihelioid membrane antigen)
Epithelioid Sarcoma and Synovial Sarcoma
IHC: MYo-D1, Myogenin+
rhabdomyosarcoma
IHC: Vimentin +
Soft tissue sarcoma
osteosarcoma translocation
unknown
11,22
ewings
ews-FL1
ewings
12,16
q13, p11
myxoid liposarcoma
TLS CHOP
Myxoid liposarcoma
2,13
alveolar rhabdomyosarcoma
pax3-FKHR
alveolar rhabdomyosarcoma
12,22
clear cell sarcoma
EWS, ATF1
clear cell sarcoma
x,18
synovial sarcoma
SSX1-SYT
synovial sarcoma
9,22
myxoid chrondrosarcoma
ews-CHN
myxoid chondrosarcoma
gene in McCune Albright syndrome
GNAS
tumor of mcune albright
polyostotic fibrous dysplasia and precocious puberty
gene of li-fraumeni
p53
tumor of li fraumeni
osteosarcoma and rhabdomyosarcoma
Fibrous displasia + soft tissue myxomas
Mazabraud syndrome
GNAS 1
multiple non ossifying fibromas, giant cell granulomas of the jaw, with cafe au lait spots
Jaffe campanacci
RECQL4 gene, sun sensitive rash with prominent poikiloderma and telengectasias, osteosarcomas
Rothmund-Thompson
Multiple enchondromas- PTHR1 gene, chonrdrosarcomas
Ollier’s dz
multiple enchondromas, angiomas, CNS, pancreatic, and ovarian malignancies
Maffucci’s
kids less than 5 Langerhans cell histiocystosis exopthalmos diabetes insipidus lytic skull lesions
Hand-Schuller-Christensen
FATAL LCH in infants
Letterer Siwe
LCH plut visceral and bone disease
Benign Enneking uses what numerus
Arabic numerals
1,2,3
Enneking malignant system uses what numerals
ROman Numerals
I, II, III
Most common stage of enneking when they present with a primary bone cancer
IIB
High grade = II
Extracompartmental -T2 and thus T1= Intra-compartmental
Mets=0, M1 is with mets
What makes something grade III enneking?
mets, distal or regional
AJCC key size for bone tumor
> 8cm
AJCC key size for soft tissue tumor
> 5cm
*ANY LESION > 5CM OR (1.5CM IN HANDS/FEET) NEEDS 3D IMAGING
BEFORE FNA/BIOPSY
NF1 PREDISPOSES TO WHICH STS
MPNST
Gardner’s familial polyposis PREDISPOSES TO WHICH STS
Low grade fibrosarcoma
radiation therapy PREDISPOSES TO WHICH STS
high grade sts
Stewart treves PREDISPOSES TO WHICH STS
Lympedema and /angiosarcoma
Dioxin (agent orange) PREDISPOSES TO WHICH STS
high grade sts
can have fluid degeneration sts
Synovial sarcoma
Biphasic cell population, epithelioid nests forming lumina and containing mucin with contrasting spindle cells running in fascicles
synovial sarcoma
fat with immature liboblasts, MDM2+, ring chromosomes
liposarcoma
marginal excision + radiation
fat juxtaposed to high grade elements
de-differentiated lisosarcoma
wide excision + radiation
herringbone pattern
fibrosarcoma
*gardner’s familial polyposis
Spindle cells in a whirling, storiform pattern, crop circles, S100+
MPNST- NF1
radiation + surgery
lymph node mets
ESARC or RACES epithelioid Synovial Angio Rhabdomyo clear cell
Most common pediatric STS
Very responsive to chemo
Rhabdomyosarcoma
epithelioid nests
spindle cells
rudimentary vascular channels
CD34+
Angiosarcoma
small round blue cells, desmin +, chromosome 2,13, PAX, FKHR
rhabdomysocarc
treatment of rhabdomyosarcooma
chemo
wide excision
+/_ radiation for + margins
MC sarcoma of hand
epithelioid
WIDE ESCISION
INVASIVE tumor, affecting multiple compartments, spindle cells in collagenous stroma, invades muscle, BETA CATENIN +, ESTROGEN RECEPTOR
FIBROMATOSIS, EXTRA ABDOMINAL DESMOID TUMOR
wide resection _ rdiation
egg on a string
schwannoma
along the nerve, follow the nerve
nuclear palisading, verocay bodies, antoni A/antoni B
Schwannoma
phleboliths
vascular malformation
sclerotherapy if small
Due to neoplastic mononuclear phagocyte
CSF1 gene
1p13
PVNS
nodular-complete excision
diffuse- complete synovectomy (typically arthroscopic, then open from back and resect if needed
rice bodies
synovial chondromatosis
excise nodules, complete synovectomy
most common sts of foot
clear cell sarcoma
synovial sarcoma
Prostaglandin that causes pain in osteioid osteoma
PGE2
continuation of osteioid osteoma, Age < 30, not relieved by NSAIDS
Osteoblastoma
Not self limiting in growth
> 2 cm in size
mixed lytic blastic
diaphyseal lesion
woven bone with NON MALIGNANT RIMMING OSTEOBLASTS
OSTEOBLASTOMA
TX OF OSTEOBLASTOMA
INTRALESIONAL EXCISION/CURETTAGE
MIRELS CRITERIA (4)
*8 or greater, ppx fixation required
Site (pertrochanteric=3)
Pain (functional pain=3)
Character/type Blastic=1, mixed=2, lytic =3
Size = >2/3 bone diameter
Low grade surface osteosarcoma with good outcomes
PAR osteal
par=stuck on bone
high grade surface osteosarcoma with intermediate outcomes
PERI osteal
treatment of parosteal osteosarcoma
wide excision
worst outcomes to best in order for the 3 types of osteosarcoma
Conventional Intramedlluary Osteosarcoma (worst)
Periosteal osteosarcoma
Parosteal osteosarcoma (best)
DIFFERNCE BETWEEN TELANGIECTATIC OSTEOSARCOMA VS ANEURYSMAL BONE CYST?
ABC DOES NOT EXPAN PAST THE PHYIS
TELANGIECTATIC CAN HAVE GIANT CELLS
IHC common in skeletal mets
Keratin
CK7 (breast and lung cancer)
TTF1 (lung cancer)
benefits of bisphosphonate therapy in mets tx
reduces rate of skeletal events (but denosumad is superior at this)
reduces lysis and associated hypercalcemia
worst heal rate of path fracture
lung-0% heal rate
Can do metastatectomy
thyroid, kidney
preoperative embolization necessary
renal cell and thyroid