Tumor Genes and Mutations

Question 1

Gene EWS-FLI1

Answer 1

Ewing’s sarcoma

t (11:22)

Question 2

PAX3-FKHR

Answer 2

Rhabdomyosarcoma
t (2:13)

Question 3

TLS-CHOP

Answer 3

Myxoid liposarcoma

t (12:16)

Question 4

SYT-SSX1

Answer 4

Synovial sarcoma

t (X:18)

Question 5

EWS-ATF1

Answer 5

Clear cell sarcoma

t (12:22)

Question 6

EWS-CHN

Answer 6

Chondrosarcoma (Myxoid Variant)

t (9:22)

Question 7

Sarcomas that metastasize to regional lymph nodes are

Answer 7

R-rhabdomyosarcoma, 
A-angiosarcoma, 
C-clear cell sarcoma.
E-epithelioid sarcoma, 
S-synovial sarcoma,

Question 8

Cat scratch Disease bug

Answer 8

Bartonella henselae. Treatment consists of azithromycin, ciprofloxacin, doxycycline

Question 9

Treatment is just Marginal Resection (4)

Answer 9

• Schwannoma/Neurilemoma (soft tissue)
• Nodular fasciitis (soft tissue)
• Epidermal inclusion cyst
• Glomus tumor

Question 10

Tx is just Wide Resection alone (5)

Answer 10

• Chondrosarcoma
• PAROsteal osteosarcoma
• Chordoma
• Adamantinoma
• Squamous cell (if no mets) (at least 5mm margin) or Mohs

Question 11

Tx is Wide Resection + Irradiation (4)

Answer 11

Soft tissue sarcoma-high grade: 
angiosarcoma, 
synovial sarcoma, 
liposarcoma, 
desmoid tumor,

Question 12

Tx is Wide Resection + Chemo(5)

Answer 12

• Osteosarcoma - Methotrexate based
• PERIosteal osteosarcoma
• Secondary sarcoma
• Dedifferentiated chondrosarcoma
• Rhabdomyosarcoma (exception to soft tissue sarcoma tx)

Question 13

Tx is Wide Resection + Chemo, +/- Radiation (2)

Answer 13

• Ewing’s - vincristine, doxorubicin, cyclophosphamide, and dactinomycin
• MFH / fibrosarcoma

Question 14

Rhabdosarcoma

Answer 14

tumor in children
• Pathology
– Small round blue cells 
– Desmin positive (muscle)
– Chromosome t(2;13) 
– PAX-FKHR gene

• Treatment VS. OTHER STS
– Neoadjuvant Chemo
– Wide excision
– +/- radiation for +margins

All STS are wide excision + Radiation (>5cm) except this one