Chapter 141 - Overview of Orthopaedic Oncology and Systemic Disease

Question 1

Bone Tumor grade

Answer 1

G1: Low grade, well differentiated (but still malignant)
- parosteal osteosarcoma
- adamantinoma
- intrameduallary, g1 chondrosarcoma
- chordoma

G2: intermediate grade, moderately differentiated
- periosteal osteosarcoma
G2 chondrosarc of bone

G3, G4: high grade, poorly differentiated or undifferentiated
- osteosarcoma
- ewing sarcoma
- undiff pleomorphic sarcoma of bone

Question 2

Enneking class of BENIGN bone tumor

Answer 2

1: inactive
- NOF
- enchondroma

2: active
- GCT
- ABC
- chondroblastoma
- chondromixoid fibroma
- unicameral bone cyst

3: aggressive
- GCT
- ABC

Question 3

Enneking class of MALIGNANT bone tumor

Answer 3

roman numerals for malignant things
a = confined to bone, no soft tissue involvement
b = extracompartmental, no soft tissue involvement

Ia: low grade intracompartmental
Ib: low grade extracompartmental

IIa: high grade, intracompartmental
IIb: high grade, extracompartmental

III: metastatic disease

Question 4

TNM stage for BONE tumors

Answer 4

based on tumor grade (low, high, metastatic), tumor size (<8cm, >8cm)

IA:
tumor grade low
<8cm

IB:
tumor grade low
>8cm

IIA:
tumor grade high
<8cm

IIB:
tumor grade high
>8cm

III:
skip lesions
any grade, any size

IV:
mets
any grade, any size

Question 5

tumor staging for SOFT TISSUE sarcoma

Answer 5

Question 6

tumor suppressor genes: p53

Answer 6

associated syndromes:
Li-fraumeni

associated tumors:
sarcomas
breast cancers

Question 7

tumor suppressor genes: Rb

Answer 7

lies on chromosome 15

associated syndromes:
hereditary neuroblastoma

associated tumor:
retinoblastoma
osteosarcoma

Question 8

tumor suppressor genes: P16INK4a

Answer 8

associated syndromes:
familial melanoma

associated tumors:
melanoma
chondrosarc
osteosarc

Question 9

tumor suppressor genes: APC

Answer 9

associated syndromes:
familial adenomatous polyposis

associated tumors:
colon adenomas
desmoid

Question 10

tumor suppressor gene: NF1

Answer 10

associated syndromes:
Neurofibromatosis

Associated tumors:
neurofibroma
sarcomas

Question 11

tumor suppressor gene: Ext1/ext2

Answer 11

associated syndrome:
multiple hereditary exostosis

associated tumor:
osteochondroma
chondrosarcoma

Question 12

chromosomal translocation: ewing sarcoma

Answer 12

t(11,22)(q24;q12)
EWS-FLI

Question 13

chromosomal translocation: synovial sarcoma

Answer 13

t(x,18)(p11;q11)
SYT-SSX

Question 14

chromosomal translocation: clear cell sarcoma

Answer 14

t(12;22)(q13;a12)
EWS-ATF1

Question 15

chromosomal translocation: alveolar rhabdomyosarcoma

Answer 15

t(2;13)
PAX3-FKHR

Question 16

chromosomal translocation: myxoid liposarcoma

Answer 16

t(12;16)
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