Pazopanib in clear cell sarcoma therapy

Abstract

Clear cell sarcoma (CCS) is a rare soft-tissue sarcoma and is characterized by a translocation t(12;22) (q13;q12). Because of some similarities to maligned melanoma it is called melanoma of soft parts. CCS has characteristic clinical features. It is commonly reported in young adults as slowly growing tumor followed by aggressive course. Its features are: regional lymph node spread and tendency for local recurrence and a propensity for pulmonary metastasis. Metastatic CCS is a very aggressive disease with pure prognosis. Conventional chemotherapy has little role to play in the management of CCS.The article presents the case of a patient with metastatic CCS with massive lung dissemination, metastatic in liver, left adrenal, pelvis and superficial soft tissue. In patient pazopanib therapy resulted in nine-month control of the disease.

Pazopanib in rare histologies of metastatic soft tissue sarcoma

“…The management of rare STS subtypes has advanced from conventional chemotherapy to oral Tyrosine Kinase Inhibitors (TKIs) targeting angiogenesis. Most data, however, come from retrospective observational studies [3,6,9,10,11,15], largely attributable to their rarity. Nevertheless, anti-vascular endothelial growth factor (anti-VEGF) TKIs like pazopanib has shown some activity in many of the rare STS subtypes [3,11,[16][17][18].…”

“…Most data, however, come from retrospective observational studies [3,6,9,10,11,15], largely attributable to their rarity. Nevertheless, anti-vascular endothelial growth factor (anti-VEGF) TKIs like pazopanib has shown some activity in many of the rare STS subtypes [3,11,[16][17][18].…”

“…Alive ( 24) Alive ( 17) In clear cell sarcoma, characterised by t(12;22) (q13;q12) [21], anthracycline-based chemotherapy has limited activity with a median PFS of 11 weeks [22]. Studies suggest activity of kinase inhibitors like Crizotinib (CREATE trial) [23], Cabozantinib targeting Mesenchymal Epithelial Transition [24] and Pazopanib targeting VEGF pathways [11]. Our study included one patient with clear cell sarcoma who achieved SD and has not progressed until the data cut-off date (Table 3).…”

“…There are only a few case reports and case series on the use of pazopanib in epithelioid sarcoma with conflicting data [8][9][10], some showing activity, and others refuting it completely. Some data are showing the activity of pazopanib in clear cell sarcoma [11].…”