MSK path VIII Flashcards
Giant cell tumor
multinucleated osteoclast type giant cells “osteoclastoma”
benign, locally aggressive
20-40 y.o
where do giant cell tumors of bone grow
epiphyseal tumor
Cx in giant cell tumor
destroyed from buldging red brown mass with cystic degeneration
soap bubble tumor
giant cell tumor
Tx giant cell tumors
curettage
mets of giant cell tumors
lungs
what drug is effective in giant cell tumors
RANKL inhibitor denosumab
aneurysmal bone cyst
tumor characterized by multiloculated blood filled cystic spaces
where do primary anerysmal bone cysts occur
first 2 decades life in metaphysis of long bones
posterior elements of vertebral bodies
common signs and Sx aneurysmal bone cysts
pain and swelling
on radiograph see eccentric expansile lesion with well defined margins, msot lytic and have thin shell reactive bone at periphery
CT and MRI show internal septa and fluid fluid levels
aneurysmal bone cyst!!
describe cysts in ABC
blood filled spaces with tan white thin septae between
“blue bone” calcified matrix
aneurysmal bone cyst ABC
Tx ABC
surgical curettage or en bloc resection
fibrous cortical defects
metaphyseal fibrous defects
common in children >2 y.o
where do fibrous cortical defects arise
eccentrically metaphysis distal femur and proximal tibia
almost half b/l or multiple
what is it called when fibrous cortical defect is 5-6 cm
nonossifying fibroma (not detected until adolescence or adulthood)
sharply demarcated radiolucencies with long axis of bone parallel to Cx
thin rim sclerosis
yellow brown cellular lesions with fibroblases and macrophages
storiform (pinwheel) formation
foamy cytoplasm
fibrous cortical defect and nonossifying fibroma
clinical course fibrous cortical defects
spontaneous resolution within several years
presentaiton of nonossifying fibroma
pathologic fracture that needs Bx and curettage
what is fibrous dysplasia
benign tumor
arise during skeletal development
monostatic fibrous dysplasia
involve one bone
polyostatic fibrous dysplasia
involve many bones
mazabraud syndrome
fibrous dysplasia and soft tissue myzomas
McCune Albright syndrome
polyostotic disease
cafe au lait skin pigmentation
endocrine abnormalities
precocious puberty!
“ground glass metaphysis”
fibrous dysplasia
morphology of fibrous dysplasia
well circumscribe, intramedullary
if large can distort bone
tan white and gritty with curvilinear trabeculae of woven bon surrounded by fibroblastic proliferation
what tumor mimics chinese characters
fibrous dysplasia
when does monostotic fibrous dysplasia occur
boys and girls early adolescence
usually stops growing when growth plate closes
bones commonly affected by monostotic fibrous dysplasia
femur, tibia, jawbones, calvarium and humerus
clinical presentation fiborus monostotic dysplasia
incidentally found
may cause pain, fracture and discrepencies in leg length
bones affected in polyostotic fibrous dysplasia
vertebrae, mandible, ulna radius, fibula, ribs, humerus, tibia, skull, femur
craniofacial involvemtn with fibrous dysplasia is seen when
when extensive skeletal disease
Tx fibrous dysplasia
orthropedic surgical procedures
bisphosphonates
can severe fibrous dysplasia transform
yes to osteosarcoma
ways of metastatic tumors to bone
direct extension, lymphatic or hematogenous dissemination, intraspinal seeding via batson plexus of veins
75% skeletal mets come from CA where
prostate, breast, kidney lung
radiographic appearance of mets tumors
lytic, blastic, mixed
prostatic adenoca mets to bone causes what
blastic lesions
carcinomas of kidney lung and GI and malignant melanoma cause what type lesions in bone
lytic
most likely CA to mets
prostate, breast, kidney, thyroid lung
what type of mutations occur in fibrous cortical defect and fibrous dysplasia
gain of function during development
