Sarcoma

Question 1

Is adjuvant chemo indicated for stage I ULMS?

Answer 1

No data to support better survival outcomes with adjuvant chemo based on observational & cohort studies and patients with low grade tumors have worse outcomes. GOG 277 compares gem+doxil ->doxil vs obs in stage 1 LMS = premature closure (low accrual) but no significant diff.

Question 2

Common mutations in ULMS

Answer 2

TP53, RB1, ATRX

Question 3

1st line tx for advanced/metastatic LMS

Answer 3

Gem/taxotere (GOG87)
-36% response rate, 6 month PFS

OR
Doxorubicin +/- ifos

• GOG250 added Bev to gem/taxotere = no improvement in OS or response rate
• GeDDiS trial: no diff in PFS it QOL when comparing gem/tax vs doxorubicin

Question 4

2nd line to LMS

Answer 4

Trabectidin
-4 mo PFS, FDA approval after anthracycline failure

• Gem/taxotere can be 2nd line if prior doxorubicin
• doxorubicin +/- ifos if gem/taxotere previously used

3rd line = trabectedin, ifos, pazopanib, eribulin, dacarbazine

Immunotherapy - has not demonstrated efficacy (ie olaratuzumab)

Question 5

Is there evidence for fertility/ovarian preservation w/ Low grade uterine sarcomas?

ESS, adenosarc (without sarcomatous overgrowth), PEComa, ole grade LMS,

Answer 5

• no definitive data
• ovarian Mets = rare
• there is data supporting safety w/ ovarian preservation in stage I LMS

-would consider it in ER+ tumors & low grade ESS

Question 6

Tx of low grade sarcomas

Answer 6

Unresectable disease

AIs
P4
Doxorubicin
Gem/tax