Sarcoma

Question 1

desmin is a marker for

Answer 1

myogenic differentiation:
RMS
leiomyosarcoma

Question 2

sarcomas are what percent of adult and pediatric tumors?

Answer 2

1% adult

13% pediatric

Question 3

s100 antigen suggests:

Answer 3

melanocytic differentiation
clear cell carcinoma
neural sheath tumors

Question 4

Potential molecular markers of sarcoma?

Answer 4

numerous well described chromosomal translocations. Think T11:22 in Ewings

Question 5

SMAC meta-analysis of adjuvant therapy for sarcoma?

Answer 5

Significant local RFS, distant RFS, overall RFS, but not for overall survival (HR 0.89 CI 0.76-1.03)

More benefit for trunkal/limb sarcomas where DOX had 7% absolute overall survival benefit at 10 years.

Question 6

Response rate of metastatic STS to single agent chemo

Answer 6

20% for doxorubicin, epirubicin and ifosfamide

PMID 7490240

Question 7

most chemo-sensitive subtypes of STS?

Answer 7

Synovial sarcomas and myxoid/round cell liposarcomas

Question 8

Do GISTs respond to standard chemotherapy?

Answer 8

No

Question 9

Additional class of chemo angiosarcomas are sensitive to?

Answer 9

taxanes (paclitaxel) but unclear if this is superior to doxorubicin/ifosfamide.

Question 10

Potential first line targeted therapy for STS (combined with Dox)

Answer 10

Olaratumab - PDGFR-alpha blocker.
FDA approved 1st line after PHASE II trial. (26.5 v 14.7 mo OS and allowed crossover)
Phase III is Announce - completes in 2020

Question 11

ASCO abstracts for Immunotherapy for STS

Answer 11

Alliance A091401 - Nivo/chemo had 6/38 response 16% http://abstracts.asco.org/199/AbstView_199_184079.html

SARC028 - pembro monotherapy - 40% response for pleomorphic sarcoma; 20% for liposarcoma. (expansion studies underway) http://abstracts.asco.org/199/AbstView_199_192958.html