Ch14 Bone Path (Part VI)

Question 1

What is the most common malignancy in the bone? Whats #2?

Answer 1

1.hematologic 2. osteosarcoma

Question 2

What are the 4 syndromes associated with osteosarcoma?

Answer 2

1. paget disease 2. Li-FrauMeni syndrome 3. Hereditary retinoblastoma 4. Rothmund-thomson syndrome

Question 3

Osetosarcoma is associated with abborhations in ____, ______, and ______

Answer 3

p53, RB1, and chromosome 21q

Question 4

What is the most common location for osteosarcoma: central, surface, or extraskeletal?

Answer 4

central

Question 5

What % of osteosarc have the SUNBURST pattern on xray?

Answer 5

25%

Question 6

Term for triangular elevation of the periostium on xray of a osterosarcoma?

Answer 6

Codman triangle

Question 7

What two stains can help differentiate a BFOL from osteosarcoma?

Answer 7

MDM2 and CDK4

Question 8

Term for a radiolucent line that corresponds to periosteum between sclerotic tumor and underlying cortex in a PAROSTEAL OSTEOSARCOMA?

Answer 8

“String sign”

Question 9

Chondrosarcoma is___ as common as osteosarcoma and ___ as common as Ewing sarcoma

Answer 9

Chondrosarcoma is ½ as common as osteosarc and 2x as common as Ewing

Question 10

Increased risk for chondrosarcoma in patients with what two chrondromatosis syndromes?

Answer 10

ollier disease, maffucci syndrome

Question 11

What is the second most common primary malignant bone tumor in pediatric patients after osteosarc?

Answer 11

Ewing sarcoma

Question 12

What is an askin tumor?

Answer 12

small round cell tumor of the chest wall (now considerd ewing sarcoma family)

Question 13

At the molecular level, Ewing sarcoma is defined by balanced chromosomal translocations that result in fusion of the RNA-binding protein \_\_\_\_ (or the closely related \_\_\_ protein) with \_\_\_ family
transcription factors (e.g., FLI1, ERG, ETV1, ETV4, and
FEV). In particular, more than 85% of cases demonstrate the translocation t(\_\_;\_\_) (q24;q12), which encodes the \_\_\_\_\_\_ fusion protein.

Answer 13

At the molecular level, Ewing sarcoma is defined by balanced chromosomal translocations that result in fusion of the RNA-binding protein EWS (or the closely related FUS protein) with ETS family transcription factors (e.g., FLI1, ERG, ETV1, ETV4, and FEV). In particular, more than 85% of cases demonstrate the translocation t(11;22) (q24;q12), which encodes the EWS-FLI1 fusion protein.

Question 14

What is the radiographic buzzterm for ewing sarcoma? (not really seen in jaw lesions, mostly extragnathic)

Answer 14

“onion skin” periosteal reaction

Question 15

What three stains can be helpful for Ewing sarcoma?

Answer 15

PAS positive granules, CD99(MIC2) +, FLI1 variable +

Question 16

What is the most common cancer involving bone?

Answer 16

Metastatic carcinoma

Question 17

What are the 5 most common primaries to met to bone?

Answer 17

1. Breast 2.Lung 3. Thyroid 4. Prostate 5.Kidney