Ms 0108 Neoplasms Of The Skeletal System

Front 1

What causes most neoplasms of the skeletal system?

Back 1

metastases

Front 2

What cancers commonly metastasize to the skeletal system?

Back 2

prostate, breast, lung, kidney, thyroid

Front 3

How do mets to the skeletal system present?

Back 3

multiple mets, patient is over 35

Front 4

Who gets primary bone cancers?

Back 4

under 35 years

Front 5

What do you use to image soft tissue neoplasms?

Back 5

MRI and CT

Front 6

What are the types of bone forming tumors?

Back 6

osteoid osteoma, osteoblastoma, osteosarcoma

Front 7

What are the types of cartilage forming tumors?

Back 7

osteochondroma, enchondroma, chondrosarcoma

Front 8

What is an osteoid osteoma?

Back 8

benign bone-producing tumor, less than 2 cm

Front 9

Who gets osteoid osteomas?

Back 9

young males

Front 10

How does osteoid osteoma present?

Back 10

severe pain, worse at night, relieved by aspirin

Front 11

What do you see on radiograph of osteoid osteoma?

Back 11

small, discrete tumor with sclerotic rim

Front 12

Which bone is usually involved in osteoid osteoma?

Back 12

diaphysis of long bones

Front 13

What does osteoid osteoma look like grossly?

Back 13

dense cortical bone with red or white central nidus

Front 14

What do you see microscopically with osteoid osteoma?

Back 14

well circumscribed nidus with randomly anastomosing bone and osteoid, rich fibrovascular stroma

Front 15

How do you treat osteoid osteoma?

Back 15

removal or radioablation of the tumor

Front 16

What is an osteoblastoma?

Back 16

looks like an osteoid osteoma histologically, but is larger

Front 17

Where do osteoblastomas occur?

Back 17

most often spine

Front 18

What are the symptoms of osteoblastoma?

Back 18

dull, achy pain, not responsive to aspirin

Front 19

How do you treat osteoblastoma?

Back 19

excision

Front 20

What is an osteosarcoma?

Back 20

malignant tumor of mesenchymal origin, produces osteoid or bony matrix

Front 21

Who gets osteosarcomas?

Back 21

10-25 yos, more often male
can also occur in patients over 40 with Pagets, radiation, or infarcts

Front 22

What is the most common symptom of osteosarcoma?

Back 22

pain associated with a mass

Front 23

Where do osteosarcomas occur?

Back 23

sites of maximal growth, distal femur, proximal tibia, proximal humerous
metaphysis is involved initially

Front 24

What do you see on radiograph of osteosarcoma?

Back 24

blastic and lytic lesions, erup through cortex, Codman's triangle

Front 25

With what is Codman's triangle associate?

Back 25

osteosarcoma

Front 26

What do you see on gross exam of osteosarcoma?

Back 26

dull gray, areas of hemorrhage and necrosis

Front 27

What do you see microscopically with osteosarcoma?

Back 27

malignant cells that make osteoid, bone, other tissues

Front 28

What is an osteoblastic osteosarcoma?

Back 28

produces mostly osteoid or bone

Front 29

What is a chondroblastic osteosarcoma?

Back 29

produces cartilage with bone

Front 30

What is a fibroblastic osteosarcoma?

Back 30

makes fibrous tissue, osteoid, bone

Front 31

How do patients with osteosarcomas present?

Back 31

1/3 with obvious mets, half with micromets, usually to the lungs

Front 32

How do you treat osteosarcomas?

Back 32

surgery and chemo

Front 33

Where do chondromas go?

Back 33

starts in small bones of hands and feet

Front 34

Who gets chondromas?

Back 34

patients in 20s-40s

Front 35

What is the prognosis of chondromas?

Back 35

benign

Front 36

What do chondromas look like on x-ray?

Back 36

lobulated or lytic

Front 37

How do chondromas present?

Back 37

asymptomatic, incidental radiographic findings

Front 38

What do chondromas look like grossly?

Back 38

cartilage, gray-blue to white and lobular

Front 39

What do chondromas look like microscopically?

Back 39

mature hyaline cartilage, can have increased cellularity, minimal atypia

Front 40

How do you treat chondromas?

Back 40

curettage, can develop pathologic fractures

Front 41

What is Ollier's disease?

Back 41

hereditary form of multiple chondromas, increased risk fo degeneration to chondrosarcoma

Front 42

What is an osteochondroma?

Back 42

bengin cartilage-capped outgrowth attached to skeleton by bony stalk

Front 43

Who gets multiple osteochondromas?

Back 43

genetic form

Front 44

How do osteochondromas present?

Back 44

incidental radiographic findings in metaphysis near growth plate in long tubular bones of young males
can have pain due to fracture or degeneration

Front 45

What do osteochondromas look like on X-ray?

Back 45

pedunculated or sessile lesion with cartilaginous cap

Front 46

What do osteochondromas look like microscopically?

Back 46

cartilage cap looks like disorganized epiphyseal plate

Front 47

What is mutliple osteochondromatosis?

Back 47

autosomal dominant, involves chromosomes 8, 11, 19, EXT 1 and 2 genes
increased risk for chondrosarcoma

Front 48

What is a chondrosarcoma?

Back 48

intramedullary malignant neoplasm, neoplastic cells make cartilage matrix without osteoid or bnoe production

Front 49

Who gets chondrosarcomas?

Back 49

40-70, uncommon in children

Front 50

Where do chondrosarcomas present?

Back 50

axial skeletal location, appear appendicularly with Ollier's and multiple osteochondromatosis

Front 51

How does the prognosis of osteosarcoma and chondrosarcoma compare?

Back 51

chondrosarcoma has a much better outcome, rare mets with chondrosarcomas

Front 52

How do chondrosarcoma mets present?

Back 52

lobulated, cortical thinning and scalloping on x-ray

Front 53

What do chondrosarcomas look like microscopically?

Back 53

disorganized cartilage, variable hypercellularity and nuclear atypia

Front 54

How do you treat chondrosarcoma?

Back 54

surgical resection with radiation

Front 55

What is fibrous dysplasia?

Back 55

benign tumor-like lesion, dysplastic disorder of bone with solitary or multifocal intramedullary lesions of fibrous cells and immature woven bone

Front 56

Where does fibrous dysplasia occur?

Back 56

ribs, femur, tibia, jawbones, skull

Front 57

What is more common, monostotic or polyostotic fibrous dysplasia?

Back 57

monostotic

Front 58

What are the most commonly involved bones in polyostotic fibrous dysplasia?

Back 58

craniofacial, pelvic, shoulders

Front 59

What is McCune-Albright syndrome?

Back 59

polyostotic fibrous dysplasia, cafe au lait spots, endocrinopathies

Front 60

What are the symptoms of monostotic fibrous dysplasia?

Back 60

mild, radiographs show "ground glass"

Front 61

How does polyostotic fibrous dysplasia present?

Back 61

progressive, with recurrent fractures, deformiteis, malignancy

Front 62

What gene is mutated in fibrous dysplasia?

Back 62

GNAS1 gene

Front 63

What is the genetic defect in malignant small round blue cell tumors?

Back 63

t11;22, produces EWS/FL-1 protein

Front 64

Who gets Ewing's Sarcoma?

Back 64

young males 10-15 yrs old

Front 65

How does Ewing's Sarcoma present?

Back 65

painful enlarging mass

Front 66

Where does Ewing's sarcoma affect?

Back 66

diaphysis of long tubular bones: femur pelvis

Front 67

What systemic symptoms are associated with Ewing's Sarcoma?

Back 67

fever, elevated sed rate, anemia, leukocytosis, can mimic infection

Front 68

What is the prognosis of Ewing's sarcoma?

Back 68

very aggressive, rapidly extends into soft tissue, metastasizes quickly

Front 69

What do you see on x-ray of Ewing's Sarcoma?

Back 69

moth-eaten lesion with periosteal reaction and large soft tissue mass

Front 70

What does Ewing's sarcoma look like microscopically?

Back 70

small and monomorphic tumor cells with "steel gray" nuclei and minimal cytoplasm

Front 71

What are the small blue cell tumors?

Back 71

lymphoma, neuroblastoma, rhabdomyosarcoma

Front 72

What is the microscopic defining characteristic of Ewing's Sarcoma?

Back 72

ltos of glycogen in cytoplasm

Front 73

What is primitive neuroectodermal tumor?

Back 73

primitive round blue cell tumor with some neural differentiation, rosette formation

Front 74

What tumor is Ewing's most related to?

Back 74

PNET

Front 75

How do you treat Ewing's?

Back 75

surgery, chemo, radiation

Front 76

How do you treat PNET?

Back 76

surgery, chemo, radiation

Front 77

What is a giant cell tumor?

Back 77

primitive mesenchymal mononuclear cellsa nd normal osteoclast-like giant cells

Front 78

Where do giant cell tumors form?

Back 78

epiphyseal location in lower femur, proximal tibia, distal radius

Front 79

Who gets giant cell tumors?

Back 79

skeletally mature young adults over 20, more females

Front 80

What is the prognosis of giant cell tumors?

Back 80

mostly benign, locally aggressive, destructive, can extend into the metaphysis

Front 81

With what mutation are giant cell tumors assocaited?

Back 81

telomeric associations, end to end fusion of intact chromosomes

Front 82

What do you see on x-ray of giant cell tumors?

Back 82

lytic epiphyseal lesions that extend to articular surface

Front 83

What do giant cell tumors look like grossly?

Back 83

cut surface is red-brown with areas of cyst formation and necrosis

Front 84

What do giant cell tumors look like microscopically?

Back 84

neoplastic "stromal" cells and reactive giant cells

Front 85

What signaling pathway is involved in giant cell tumors?

Back 85

RANK/RANKL: receptor activator of nuclear kappa B ligand

Front 86

How do you treat giant cell tumors?

Back 86

curettage

Front 87

What is an aneurysmal bone cyst?

Back 87

primary or secondary benign cystic lesions of bone

Front 88

Who gets aneurysmal bone cysts?

Back 88

people in their 20s

Front 89

Where do aneurysmal bone cysts affect?

Back 89

distal femur, proximal tibia, spine

Front 90

What do aneurysmal bone cysts look like?

Back 90

fibrous cyst wall with giant cells and reactive bone formation

Front 91

What mutation is present in aneurysmal bone cysts?

Back 91

16q22 and 17p13

Front 92

How do you treat aneurysmal bone cysts?

Back 92

curettage