Sarcoma treatment - where are we now

Incidence of sarcomas estimated to have to have hit 36,000 in 2008 in the seven major markets - Treatment of GIST represents a success story in oncology while significant unmet needs remain for other sarcomas

Sarcomas are a diverse group of rare connective tissue tumors representing only 1% of all adult and 15% of all childhood cancers, the development of metastatic disease is a major clinical problem particularly for soft tissue sarcomas.

Treatment depends mainly on the sarcoma subtype. For example, advanced gastrointestinal stromal tumors (GIST) are primarily managed using molecular-targeted therapies, whereas other soft tissue sarcoma subtypes and bone sarcomas are managed using conventional chemotherapy with surgery and/or radiotherapy.

In a just released report on the current state and future of sarcoma treatment it was concluded that better classification and understanding of disease mechanisms may facilitate a shift towards the use of more novel molecular-targeted therapies.

The treatment of GIST represents one of the success stories in modern day oncology.

The identification of specific mutations that are key drivers of GIST has permitted the use of two effective small molecule tyrosine kinase inhibitors: Gleevec/Glivec (imatinib; Novartis) and Sutent (sunitinib; Pfizer).

Nevertheless, the development of tyrosine kinase-resistance in Gleevec-treated GIST patients has created opportunity for more novel molecular-targeted therapies to enter the market. These include Novartis’s second-generation Tasigna (nilotinib) for third line GIST, Genentech/Roche/Chugai’s Avastin (bevacizumab) for first-line GIST and Infinity/MedImmune’s Hsp90 inhibitor retaspimycin for third-line GIST.

As the manufacturer of the current standard first line GIST drug Gleevec, Novartis is perfectly positioned to ensure a potentially smooth and high impact entry of Tasigna into the third-line GIST market.

Improved treatments of unresectable, metastatic bone and soft tissue sarcomas (excluding GIST) represents a key unmet need. Limited therapeutic advances have been made in the treatment of this oncology sub-class. One such agent in Phase III development for metastatic soft tissue and bone sarcomas is ARIAD/Merck & Co’s oral mTOR (mammalian target of rapamycin) inhibitor deforolimus, which has the potential to improve the currently unchanged survival of metastatic sarcoma patients.

Read more about current options, unmet needs and pipeline candidates in sarcoma here