431P - Alveolar soft part sarcomas: A tertiary care Indian centre experience

Background

Alveolar soft part sarcoma (ASPS) is an ultra-rare and chemo refractory sarcoma. Tyrosine kinase inhibitors (TKI) are promising however, feasibility data is sparse from low-middle income countries.

Methods

ASPS patients registered in our institute from year 2001-2021 were analysed.

Results

There were 85 patients with a median age of 32 years; 48 (57 %) were males, commonest primary site was extremities in 57(67%), 31 (37%) were de-novo metastatic(mASPS). Among the 54 (63%) non-metastatic patients, 24 relapsed and became metastatic and hence 55(65%) patients were treated for their metastatic disease at some point. Of the 55, 23 (41%) received best supportive care (BSC), 10 (18 %) received TKI (7- pazopanib, 3- sunitinib) and remaining received other systemic therapies;8 (80%) had clinico-radiological responses, including 5(50%)-partial responses and 3(30%) stable disease. At a median follow up of 18(95%CI8-26) months, median event (progression/relapse/death) free survival(EFS) was 24(95%CI 2-45) months and 3 and 5 year predicted EFS were 40% and 21% respectively.r. The median EFS among those treated with TKI versus BSC arm were 50 (95% CI0-108) months and 20 (95% CI 0-42) months, p =0.045, HR0.27(95%CI 0.06-1) respectively. After progression on one TKI, 5 were re-challenged with another TKI and had a median time to progression of 3.5 months. The TKI were well tolerated with significant grade 3/4 toxicities seen (with 800 mg dose) in 3 patients including diarrhea (3), mucositis (1), hyponatremia (1). De-escalation to 600 mg leads to good tolerance in 7(70%). few patients only visited for second opinion and advised TKI however, for them further details are not available. Among the 54 nmASPS, 41(76%) underwent surgery, 34 (63%) received adjuvant and 4 received definitive radiation, 2 received adjuvant chemotherapy(ifosfamide-adriamycin). At a median follow up of 27(95% CI-9-46) months, median EFS was 46(95% CI 0--142) months and predicted 3& 5-year EFS were 54%& 47% respectively.

Conclusions

The large series in ASPS suggest comparable practice pattern and results to published literature in nmASPS. There was a transition in treatment approach for mASPS from BSC towards TKI with good clinical benefit and acceptable tolerance and is a feasible standard of care option to mASPS.

Clinical trial identification

Editorial acknowledgement

Legal entity responsible for the study

The authors.

Funding

Has not received any funding.

Disclosure

All authors have declared no conflicts of interest.