Understanding CIC rearranged sarcoma

The Australia New Zealand Sarcoma Association (ANZSA) has recently published a paper to assess the treatment pathways and outcomes of CIC-rearranged sarcoma.

CIC-rearranged sarcoma is a newly established sarcoma subtype.  It is very rare which makes it hard to know how best to classify and risk stratify this subtype.

Typically this sarcoma subtype presents in younger adults (25-35 years) and unfortunately the sarcoma is aggressive and linked to a poor outcome.

This retrospective study is the first clinical series to comprehensively detail individualised systemic treatment use and to summarise available literature for CIC rearranged sarcoma.

The median overall survival was 16.3 months with the 2- and 5-year overall survival rate of 44% and 24% respectively.  In those with advanced disease the median overall survival was 12.6 months.  There is a high rate of recurrence as patients tend not to be chemo-sensitive.

In this analysis the usual management for localised disease was surgical resection, chemotherapy with Ewing-based regimens, and adjuvant radiation.

Now we understand more about this new subtype, research is needed to establish optimum treatment approaches for both localised and advanced disease.   ANZSA is looking to collaborate internationally to further research.  

Please donate to help important research into this rare subtype.

To read the findings in the paper click here