Uterine Tumor Resembling Sex-Cord Tumors of the Ovary (Utrosct): A Case of Long-Term Response to Pazopanib

Background: uterine tumor resembling sex-cord tumors of the ovary (UTROSCT) is a rare form of mesenchymal uterine tumor of uncertain malignancy. Sporadic cases of advanced disease are reported, but little is known about its management in the metastatic setting. Pazopanib is a multi-tyrosine kinase inhibitor (TKI), approved for the treatment of advanced renal cell carcinoma and advanced soft tissue sarcomas, which affects several targets, including VEGFR1-2-3, PDGFRα-β and FGFR1-3. Case presentation: here we report a case of metastatic UTROSCT with strong and prolonged clinical response to pazopanib. Conclusions: these findings suggest that this anti-angiogenic approach should be considered in planning future therapeutic strategies for this rare disease. Furthermore, we noted the importance of including patients with rare diseases in clinical trials and investigating molecular pathogenesis.