Sarcoma

Question 1

Myxoid liposarc features?

Answer 1

CHOP translocation
Younger patients
Extremities
Spine mets
Radiation sensitive

Question 2

Leiomyosarcoma features?

Answer 2

RB 1 associated, Li Fraumeni
Intraabdominal most common
High distant met risk
Radiation not useful per STRASS
Dacarbazine, Doxorubicin

Question 3

MPNST?

Answer 3

NF1 associated 40%
Radiation associated 10%(use with caution in NF1 patients)
Proximal large nerves (sciatic most common
Use radiation adjuvant

Question 4

DFSP?

Answer 4

17:22 translocation PDGF (COL1A1-PDGFB fusion)
Trunk, Very slow growing
High LR, WLE 2cm with radiation tx
Fibrosarcomatous variant aggressive, can metastasize
TKI effective

Question 5

Solitary fibrous tumor

Answer 5

Fusion product NAB2-STAT6
Doege Potter syndrome (hypoglycemia due to IGF)
Pleura then peritoneum
Slow growing with low distant mets

Question 6

Myxofibrosarcoma

Answer 6

Most common sarcoma among elderly
Usually limbs
From dermis or subcutaneous
Infiltrative pattern, highest LR rate
Very wide excision (2-5 cm)
Radio resistant, sometimes used adjuvant
Less myxoid more aggressive
Epitheloid myxofibrosarc high LRR and distant met rate

Question 7

Synovial sarcoma

Answer 7

Young patients
SYT-SSX fusion
Proximity to joints
Very aggressive, high mets
Radiosensitive

Question 8

Angiosarcoma

Answer 8

Old people, scalp extremities
Purplish nodule
Radio and chemosensitive (paclitaxel)
Wide excision
Can happen post radiation (7 yrs median) or with chronic lymphedema