MSK path VIII

Question 1

Giant cell tumor

Answer 1

multinucleated osteoclast type giant cells “osteoclastoma”
benign, locally aggressive
20-40 y.o

Question 2

where do giant cell tumors of bone grow

Answer 2

epiphyseal tumor

Question 3

Cx in giant cell tumor

Answer 3

destroyed from buldging red brown mass with cystic degeneration

Question 4

soap bubble tumor

Answer 4

giant cell tumor

Question 5

Tx giant cell tumors

Answer 5

curettage

Question 6

mets of giant cell tumors

Answer 6

lungs

Question 7

what drug is effective in giant cell tumors

Answer 7

RANKL inhibitor denosumab

Question 8

aneurysmal bone cyst

Answer 8

tumor characterized by multiloculated blood filled cystic spaces

Question 9

where do primary anerysmal bone cysts occur

Answer 9

first 2 decades life in metaphysis of long bones

posterior elements of vertebral bodies

Question 10

common signs and Sx aneurysmal bone cysts

Answer 10

pain and swelling

Question 11

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

Answer 11

aneurysmal bone cyst!!

Question 12

describe cysts in ABC

Answer 12

blood filled spaces with tan white thin septae between

Question 13

“blue bone” calcified matrix

Answer 13

aneurysmal bone cyst ABC

Question 14

Tx ABC

Answer 14

surgical curettage or en bloc resection

Question 15

fibrous cortical defects

Answer 15

metaphyseal fibrous defects

common in children >2 y.o

Question 16

where do fibrous cortical defects arise

Answer 16

eccentrically metaphysis distal femur and proximal tibia

almost half b/l or multiple

Question 17

what is it called when fibrous cortical defect is 5-6 cm

Answer 17

nonossifying fibroma (not detected until adolescence or adulthood)

Question 18

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

Answer 18

fibrous cortical defect and nonossifying fibroma

Question 19

clinical course fibrous cortical defects

Answer 19

spontaneous resolution within several years

Question 20

presentaiton of nonossifying fibroma

Answer 20

pathologic fracture that needs Bx and curettage

Question 21

what is fibrous dysplasia

Answer 21

benign tumor

arise during skeletal development

Question 22

monostatic fibrous dysplasia

Answer 22

involve one bone

Question 23

polyostatic fibrous dysplasia

Answer 23

involve many bones

Question 24

mazabraud syndrome

Answer 24

fibrous dysplasia and soft tissue myzomas

Question 25

McCune Albright syndrome

Answer 25

polyostotic disease cafe au lait skin pigmentation endocrine abnormalities precocious puberty!

Question 26

"ground glass metaphysis"

Answer 26

fibrous dysplasia

Question 27

morphology of fibrous dysplasia

Answer 27

well circumscribe, intramedullary if large can distort bone tan white and gritty with curvilinear trabeculae of woven bon surrounded by fibroblastic proliferation

Question 28

what tumor mimics chinese characters

Answer 28

fibrous dysplasia

Question 29

when does monostotic fibrous dysplasia occur

Answer 29

boys and girls early adolescence | usually stops growing when growth plate closes

Question 30

bones commonly affected by monostotic fibrous dysplasia

Answer 30

femur, tibia, jawbones, calvarium and humerus

Question 31

clinical presentation fiborus monostotic dysplasia

Answer 31

incidentally found | may cause pain, fracture and discrepencies in leg length

Question 32

bones affected in polyostotic fibrous dysplasia

Answer 32

vertebrae, mandible, ulna radius, fibula, ribs, humerus, tibia, skull, femur

Question 33

craniofacial involvemtn with fibrous dysplasia is seen when

Answer 33

when extensive skeletal disease

Question 34

Tx fibrous dysplasia

Answer 34

orthropedic surgical procedures | bisphosphonates

Question 35

can severe fibrous dysplasia transform

Answer 35

yes to osteosarcoma

Question 36

ways of metastatic tumors to bone

Answer 36

direct extension, lymphatic or hematogenous dissemination, intraspinal seeding via batson plexus of veins

Question 37

75% skeletal mets come from CA where

Answer 37

prostate, breast, kidney lung

Question 38

radiographic appearance of mets tumors

Answer 38

lytic, blastic, mixed

Question 39

prostatic adenoca mets to bone causes what

Answer 39

blastic lesions

Question 40

carcinomas of kidney lung and GI and malignant melanoma cause what type lesions in bone

Answer 40

lytic

Question 41

most likely CA to mets

Answer 41

prostate, breast, kidney, thyroid lung

Question 42

what type of mutations occur in fibrous cortical defect and fibrous dysplasia

Answer 42

gain of function during development