Treatment of Multidrug Resistant Advanced Alveolar Soft Part Sarcoma With Sunitinib

Abstract

Sunitinib is a tyrosine kinase/angiogenesis inhibitor with proven efficacy in gastrointestinal stromal tumor and advanced renal cell carcinoma. We are presenting the case report of a patient with aggressive alveolar soft part sarcoma with lung and bone metastases, who had failed multiple chemotherapy regimens showing significant response to sunitinib. There was not only complete regression of the primary tumor, stabilization of his bone metastases and significant improvement in the quality of life. Our report shows that sunitinib has the capability of playing a pivotal role in the management of non-gastrointestinal stromal tumors like alveolar soft part sarcoma. Further research and trials must be encouraged over the use of this drug as it is most definitely promising.

Alveolar soft part sarcoma in children and young adults: A report of 69 cases

“…Therefore, due to the pathophysiology and vascular nature of this tumor, novel therapeutic agents such as tyrosine kinase receptor inhibitors or antiangiogenic therapy are considered a rational approach for this disease . Recently, agents such as cediranib, sunitinib, dasatinib, pazopanib, and tivantinib have been used in ASPS with promising results. In Table , we summarize the results of reports regarding targeted therapy for ASPS including the current series .…”

Paediatric and adolescent alveolar soft part sarcoma: A joint series from European cooperative groups

“…One out of 14 patients with grossly resected tumour at diagnosis (IRS-I or II) treated with chemotherapy had a metastatic relapse versus 3 out of 14 who did not receive any adjuvant chemotherapy. Other drugs in ASPS may have an anti-angiogenesis effect, such as bevacizumab [9], sunitinib [10][11][12] or interferon alpha-2b [20]. Phase II trials with cediranib and a c-Met inhibitor (ARQ-197) have recently confirmed the efficacy of these drugs [8,21].…”

“…Results. Median age was 13 years [range: [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. Primary sites included mostly limbs (63%).…”

“…sunitinib, cediranib or bevacizumab) have been shown to be effective in adult ASPS [8][9][10][11]. Although the extreme rarity of ASPS in children precludes exclusively paediatric studies on this specific histotype, recent data indicate that paediatric cases might be equally sensitive to new targeted therapies [11,12], but further data are needed to confirm this finding.…”

Tivantinib (ARQ 197), a selective inhibitor of MET, in patients with microphthalmia transcription factor–associated tumors

“…Several groups have recently reported responses in MiT tumors treated with multitargeted kinase inhibitors that block vascular endothelial growth factor receptor 2 (VEGFR2). Decreases in tumor dimensions and/or density were reported in ASPS and CCS treated with sunitinib37‐40 or cediranib 41. Moreover, minor responses have been reported in a patient with ASPS treated with bevacizumab,42 and both sunitinib and sorafenib have been reported to have activity in patients with tRCC 43, 44.…”