Desmoid tumor (DT) management in dedicated sarcoma medical oncology clinic in a developing country: Lessons learnt.

Abstract

e22541 Background: There is lack of data about desmoid tumors from India except case reports. It is not known how these patients are dealt with outside tertiary care centres who lack expertise. Methods: We retrospectively analysed all patients of DT from a prospectively maintained database who were referred to AIIMS Sarcoma Medical Oncology Clinic from January 2016 till 2019. Results: There were total of 56 patients with male: female ratio of 3:4. Median age was 26 (3-57) years. Extremity was most common site (57%) and median tumour size was 12 (range 3-20) cm. One patient (1.8%) had FAP and 1 (1.8%) had multifocal disease. A total of 27 patients received first treatment outside our institute and was primary surgery in all (100%). However, of the remaining 29 patients who primarily presented to us, surgery was done only in 3 (10%) because of patients choice and pelvic location. Five patients were kept under observation and all are doing well. Tamoxifen with celecoxib was most common first line therapy in 80% (40/50) with other options being sorafenib (6/50) and chemotherapy (4/50). Chemotherapy was used in abdomen (3/4) and head neck (1/4) primary patients. Menstrual irregularities including amenorrhoea was most common toxicity in 33% (13/40) with tamoxifen combination and a cause of concern in this cohort of young females leading to discontinuation of therapy inspite of SD in 54% (7/13) patients. Second line therapy was started in 20 patients with sorafenib being most common (13/20), others being chemotherapy (4/20), tamoxifen combination (2/20) and imatinib (1/20). Of all 19 patients on sorafenib, 10 (53%) experienced Hand Foot Syndrome. None discontinued sorafenib due to HFS. After median follow up of 15 months, median progression free survival (PFS) with first line was 29 months (11-46) and with second line it was not reached. There have been three deaths till date (5.3%), all were non-extremity primaries. Conclusions: In Indian setup, majority of patients presenting outside tertiary care centre undergo surgery as the first line therapy without any inclusion of medical therapy. This might be attributed to lack of awareness about medical therapy and natural trajectory of disease. Tamoxifen though was a commonly used option, led to toxicity which is of concern in young Indian patients leading to discontinuation. Sorafenib is well tolerable except HFS but longer follow up is needed for efficacy analysis.