Dougherty Part 4 Flashcards
chondrosarcoma by definition has no what
malignant bone formation
what are the types of chondrosarcomas based on calcificaiton of location
intramedullary
juxtacortical and surface
what are the types of chondrosarcoma classified histologically
conventional (hyaline and/or myxoid)
clear cell
dedifferentiated
mesenchymal
chondrosarcoma is seen in what ages
over 40
clear cell and mesenchymal occur in teens
where does chondrosarcoma affect the skeleton
pelvis, shoulder, ribs
15% of chondrosarcoma arise in what
enchondroma (higher risk with multiple enchondromas, and osteochondroma
where is clear cell chondrosarcoma found
long tubular bone epiphyses
what is seen on an X-ray of conventional chondrosarcoma
nodular growth with endosteal scalloping and flocculent densities from calcification
reactive thickened cortex bone
where does conventional chondrosarcoma metastasize to
lungs
bone
what is dedifferentiated chondrosarcoma
low-grade chondrosarcoma and high grade sarcoma
clear cell chondrosarcoma is found in what patients and where
teens/young adults
epiphysis
mesenchymal chondrosarcoma is found in what patients
teens/young adults
fibrous cortical defect affects what age group
40% of children
nonossifying fibroma is found in what age group
adolescent
where can one find fibrous cortical defects
metaphysis/cortex
knee
where can one find non ossifying fibroma
metapysis/cortex
knee
what is the size of a fibrous cortical defect
small (less than 0.5cm)
what is the size of a non ossifying fibroma
large up to 6cm
fibrous dysplasia shows what 3 patterns
monostotic
polyostotic with soft tissue myxomas
polyostotic with endocrine dysfunction (McCune-Albright Syndrome)
what is seen in McCune-Albright Syndrome
cafe aurait skin pigmentations
endocrine: sexual precocity (often presenting), hyperthyroidism, pituitary adenomas with GH, primary adrenal hyperplasia
hyperactive G-protien of GNAS gene
McCune-Albright Syndrome
McCune-Albright Syndrome is seen in what
fibrous dysplasia
Chinese character woven bone in fibrous stroma is seen in what
fibrous dysplasia
what is seen in fibrous dysplasia
well-circumsctibed intramedullary
large may expand and distort bone
gross: tan-white and gritty
80% of Ewing Sarcoma is in what age group
less than 20 years
what is ewing sarcoma
small round (blue) cell tumor of bone and soft tissue
glycogen
Homer-Wright Rosettes
what is the issue in ewing sarcoma
EWS gene translocation
t(11;22) EWS-FLI1
what is the difference between Ewing Sarcoma and Primative Neuroectodermal Tumor (PNET)
degree of neural differentiation
where on the bone and in what bones is ewing sarcoma and PNET
diaphysis of long tubular bones (FEMUR) and flat bones of pelvis
when would on see lytic destruction onionskin periosteal reaction and sunburst pattern
Ewing sarcoma
PNET
what is Giant Cell Tumor of Bone
benign locally aggressive tumor of macrophage/monocyte system
giant cell tumors are normally found where in the bone
epiphyses but may extend into the metaphysis
where are giant cell tumors most commonly found
knee
in giant cell tumors mononuclear cells express what
RANKL
grossly what does one see in giant cell tumor of bone
brown from hemosiderin
what is seen in Aneurysmal Bone Cyst (ABC)
pain and swelling
multiloculated blood-filled cysts
rapid growth may appear malignant
aneurysmal bone cyst (ABC) is common in what ages
below 20
where and in which bones is aneurysmal bone cyst (ABC) found
metaphysis
long bones
vertebrae
what histological features are seen in Aneurysmal Bone Cyst
blood filled cysts separated by internal septae
fibroblasts and multinucleate giant cells
woven bone
what is the most common form of skeletal malignancy
metastatic disease
what is the pathway of spread in metastatic disease
direct extension
lymphatic or hematogenous
intraspinal seeding (Baston plexus of veins)
adult metastatic disease is commonly from where
“BLT and a Kosher Pickle”
Breast, lung, thyroid, kidney, prostate
metastatic disease is most often found where
axial skeleton
osteoblastic metastases in the vertebral column is most likely from where
patient with metastatic adenocarcinoma of prostate
what are peidatric metastatic diseases
Neuroblastoma Wilms Tumor Osteosarcoma Ewing sarcoma Rhabdomyosarcoma