Bone Tumors

Question 1

How do bone tumors usually present?

Answer 1

nonspecific!

pain (worse at ngiht)

mass

pathologi fracture

aymptomatic

Question 2

What ae the diagnostic factors from bone tumors?

Answer 2

• age
• sex
• skeletal location
  
  • specific bone
  • specific area of bone
    
    • medullary cavity, cortex, juxtacortical
    • epiphysis, metaphysis, diaphysis

Question 3

What bone tumors are common in

children?

young adults?

elderly?

Answer 3

Children, adolecents: osteosarcoma, Ewing’s sarcoma

Young adults: Giant cell tumor

Elderly: Chondrosarcoma

Question 4

What is a sclerotic (well-defined) margin in a radiologic pattern typically an indication of?

Answer 4

a benign, slowly growing neoplasm

Question 5

what is an illdefined margin radiologically typically an indication of?

Answer 5

malignant, rapidly growing neoplasm

Question 6

hat is a solid ivory pattern typically and indicator of?

Answer 6

malignant bone-matrix forming tumors

Question 7

what are rings and arcs typically an indicator of

Answer 7

chondroid matric fomring tumors

Question 8

what are 2 benign bone forming tumors and what are the differences in type of bone, size, pain and response to aspirin

Answer 8

• Osteoid Osteoma
  
  • long bones, femur and tibia
  • less than 2cm
  • night pain
  • responds to aspirin
  • radiolucent lesion within sclerotic cortex
• Osteoblastoma
  
  • vertebrae or long bone metaphysis
  • more than 2 cm
  • painful
  • not responsive to aspirin
  • expansile radio-lucency with mottling

Question 9

what is this?

Answer 9

Osteoid osteoma

central area of immature bone formation. they are all osteoblasts that are producing osteoid that is becoming poorly formed bone

Question 10

What is this?

Answer 10

osteoid osteoma- note the osteoblasts look normal!!

Question 11

WHat is this?

Answer 11

osteoblastoma

• histologically siilar to osteoid osteoma
• circumscribed benign lesion in bone
• look like osteoid/osteoma
• area of immature bone being formed by osteoblats

Question 12

What are bone-forming (osteogenic) tumors?

Answer 12

• rare group of tumors in comparison with carcinomas and hematopoietic tumors bc malignant bone tumor comprise 0.2% of all types of cancers
• represent an important percentage of potentially curable cancers following a multimodal therapy

Question 13

Define the pathogenesis of an osteosarcoma:

Answer 13

• inherited mutant allele of Rb gene (hereditary Rb: marked increase (1000x) in OS
• mutation of p53 supressor gene
  
  • Li-FraumeniL bone and soft tissue sarcomas, easryl onset breast cncer, brain tumors, leukemia
• Over expression of MDM2 (5-10%) INK4 and p16
• site of bone growht/disease (is pagez)
• prior irradiation

Question 14

What is an osteosarcoma and what is the incdence and age distribution

Answer 14

• malignant mesenchymal tumor in which cells produce osteoid or bone
• 2000 new a year
• mos tcommon sarcoma of bone
• bimodal age distribution: M>F mean age is 15 2nd peak is 55-80

Question 15

Where does an osteosarcomea usually ocur? where is spread common

Answer 15

• metaphysis of long bones
  
  • femur, tibia, humerus, flat bones, spine (sometime polyostotic but usually not)
• Hematogenous spread to lungs is common (if not treated will happen for sure)

Question 16

what are some characteristic of an osteosarcoma?

Answer 16

• poorly delineated
• bone destruction
• cortical disruption
• bone matrix
• soft tissue extension
• codman’s triangle (bone trying to make new cortex around the tumor)

Question 17

What is the pathology of an osetosarcoma and what is the treatment?

Answer 17

• pathology: infiltrative tumor, extending into soft tissue, malignant cells producing osteoid
• treatment: neo-adjuvant chemotherapy and surgical resection
• prominent irregularly shaped nuclei a lot of mitotic activity that are often atypical, pleomorphism seen histologically

Question 18

what is the prognosis of an osteosarcoma?

Answer 18

• post chemo: 60-65% 3-5 yr survivl for pts with non-metastatic disease
• En bloc resection following chemotherapy: >90% necrosis which leads to near 90% survival

Question 19

What is an osteochondroma?

Answer 19

• the most common benign tumor of bone
• metaphysis of long bones
• malignancy is rare but increased risk in herditary multiple extoses
  
  • autosomal dominant, usualyl EXT-1 (8q24)
• small growth that comes off the cortex with a cartilagenous cap

Question 20

What is this?

Answer 20

where the cartilage cap is forming just looks like normal cartilage!

Question 21

What is an enchondroma?

Answer 21

benign hyaline cartialge lesion

• enchondroma: intramedullary chondroma
• periosteal chondroma: juxtachortical chondroma (ie located on the cortica surface under the periosteum)

Question 22

How do you diagnose and what is the treatment for enchondroma?

Answer 22

• usually aymptomatic, incidental, appendicular skeleton; small bones of hands and feet
• X-rays: lytic, lobulated, cortical thinning
• micro: lobules of hyaline cartilage, minimal typia
• treatmentL none, unless lesion shows change:
  
  • symptomatic (onset of acute pain is felt to be circumstantial evidence that lesion is malignant)
  • evidence of recent growth after skeletal maturity

Question 23

what does and enchondroma look lik ehistologically?

Answer 23

typical chondrocytes in the lacunae, looks bland and benign

Question 24

What point mutations adn diseases are frequently associated wth mutliple chondromatosis?

Answer 24

frequent point mutations in IDH1 or IDH2

• Ollier disease
• Maffucci syndrome

Question 25

What is ollier disease

Answer 25

point mutation in IDH1 or IDH2, multiple chondromatosis * multiple enchondromata * tend to have regional distribution * +/- severe skeletal malformation

Question 26

What is Maffucci Syndrome

Answer 26

frequent poitn mutations in IDH1 or IDH2 * multiple echondromata and agiomata * svere skeletal malformation * higher incidednce of malignant transformation

Question 27

what is a chondrosarcoma?

Answer 27

malignant tumor in which neoplastic cells produce a purely catilagenous matrix second most common bone sarcoma (26% of bone sarcomas) mainly older adults, but wide age range central skeleton: **pelvis** and ribs, jumerus, femur

Question 28

What is seen with a chondrosarcoma on imagin?

Answer 28

* medullary location * frequent ly ontain calcifications, which tend to be lost in grade 3 tumors * cortical erosions or destruction (pop-corn like) rings and arcs bc cartilage. look swhite.blusih bc cartilage * occasional soft tissue extension

Question 29

what is the pathology of chrondrosarcoma?

Answer 29

* generally more cellular and nuclei more pleomorphic than in enchondromas * binucleation is frequent, but does not siffice for malignant diagnosis * myxoid chaneg of chondroid matrix \*\*so remember endochondromas the chondrocytes looked pretty normal and there was a normal amount, now ther are way more and they look atypical and tehre are mitosis

Question 30

in a chondrosarcoma ______ and \_\_\_\_\_\_\_correlate with behavior? also what is grading based on?

Answer 30

in chondrsarcoma size and grade correlate with behavior, also grade is based on degreee of cellularity and atypia

Question 31

Chondrosarcoma grade 1

Answer 31

more cellular than enchondroma but more matrix than cells, still some atypia, but not much cells look fairly normal

Question 32

Chondrosarcoma grade 2

Answer 32

more atypia with some focal areas that are dense with cellularity. areas of clear cut cartilage expression

Question 33

chondrosarcoma grade 3

Answer 33

more severe atypia with mitoses more aggressive and fairly rare

Question 34

how does 5 year survival relate to grade for a chondrsarcoma? what are the variants?

Answer 34

* higher grade=lower 5 yerr survival * grade 1,2: 80-90% * grade 3: 29% (pulmonary metastasis) * Variants: de-differentiated, myxoid, clear cell, mesenchymal, juxtacortical

Question 35

What is a non-ossifying fibroma? location and age? How is ti usually identitied

Answer 35

* common developmental cortical defect * most comm space-occupying lesion of bone: 1/4, often multifocal * tibia femur (metahysis) 1-3 decades * eccentric lytic, peripheral sclerosis * incidental finding or pathologic fracture

Question 36

what pattern does a non-ossifying fibroma have?

Answer 36

storiform pattern

Question 37

What is fibrous dysplasia?

Answer 37

* developmental arrest of bone * monostotic: most common, seen in adolecents, ribs manidble femur * polyostotic: infancy/childhood, crippling deformities, craniofacial involvement common

Question 38

What is McCune-Albright Syndrome?

Answer 38

a fibrous dysplasia * polyostotic FD with endocrinopathies and cafe-au-lait spots * rare-form; F.M * sexual precocity, acromegaliy, cushing syndrome * activating germline mutations of GNAS (GTP-binding protein) results in excess cAMP leading to endocrine gland hyperfunction

Question 39

what are 5 features of a fibrous dysplasi?

Answer 39

expansible circuscribed thinned cortes "ground glass" may be multiple

Question 40

what is the pathology of fibrous dysplasia? What s the treatment?

Answer 40

* haphazard, curvilinear, randomly oriented woven bone trabeculae ("chinees characters") surrounded by fibroblastic stroma * no significant osteoblastic rimming * (bone present in fibrous tissue) * treatment: conservative except for is polyostotic then you scrape it out!

Question 41

What is the incidence of Ewing Sarcoma/PNET? How does it present and where does it occur?

Answer 41

2nd most common malignant bone tumor in childhood * adolecents, young adults M\>F * present as painful, often enlargin mass * **diaphysis** of long tubular bones, ribs and pelvis

Question 42

What is seen on X-ray with an Ewing-Sarcoma

Answer 42

destructive moth-eaten, premeative lesion with large soft tissue mass onion skin pattern of periosteal reaction in response to rapid growth

Question 43

What is the pathology of Ewing Sarcoma?

Answer 43

* sheets of primitve small roung blue cells with neural phenotype * membranous CD99 * Contain abundant glycogen * hemorrhage and necrosis common

Question 44

What is the pathogenesis of Ewing Sarcome/PNET?

Answer 44

* EWS involve in over 95% of ES/PNET * translocation: t(11;22) present in 85% of tumors * EWS on 22q fused with FLI-1 transcription factor on 11q

Question 45

Treatment of Ewing Sarcome/PNET? and 5 year survival for stage 1?

Answer 45

chemotherapy and surgery radiation may be added stage I: 5 year survival 70% with chemo/RT

Question 46

Who gets Giant Cell Tumor of Bone?

Answer 46

young adults (20-4-yo), older adolesecents (skeletally matured) females\>males

Question 47

Where do Giant cell tumors of the bone occur?

Answer 47

epiphyseal location: knee, proximal humerus, radius most are benign, locally aggressive but may destroy cortex of bone and extend into soft tissue

Question 48

What is seen histologically with a giant cell tumor of bone?

Answer 48

multinucleate giant cells, that look like osteoclasts

Question 49

what is the most common malignant bone tumor especially in adults (20x more frequent that primary bone tumors)

Answer 49

metastatic bone tumors

Question 50

What are metastatic bone tumors like?

Answer 50

* mostly multiple * solitary lesions may mimic a primary bone tumor and precede discovery of its source * 70% go to axial skeleton (skull, ribs, vertebral colum, sacrum) * mostly lytic * may be blastic (bone-forming): breast, prostate * 80% breast, lung, thryoid, prostate, and kidney (BLT-KP)

Question 51

what is the most common sarcoma in the bone?

Answer 51

osteosarcoma

Question 52

what age raneg doe sosteosarcoma typically occur in?

Answer 52

children/adolescents

Question 53

what is osteoid osteoma

Answer 53

painful bone lesion that is releived by aspirin

Question 54

Li-Fraumeni syndrome, Ollier disease, Mafucci syndorme, and McCune-Albright syndrome are all associted with \_\_\_\_\_\_\_\_

Answer 54

bone lesions