case_images: PMC8032946_01
case_images: Initial pathology.
case_images: fonc-11-641376-g0001.jpg
case_images: PMC8032946_01_fonc-11-641376-g0001.jpg
case_images: F1
case_images: Both bone biopsy and hysterectomy indicated an oligo-metastatic uterine PEComa, without cervical involvement, with high-grade nuclear features and lymphovascular invasions (Figure 1).
case_images: NGS 400 genes.
case_images: fonc-11-641376-g0002.jpg
case_images: PMC8032946_01_fonc-11-641376-g0002.jpg
case_images: F2
case_images: Targeted NGS (52-cancer gene hotspot panel) detected no mutation but a heterozygous deletion of 9q34 that contains the TSC1 gene (Figure 2), prompting a first line everolimus treatment (10 mg 1x/day, po).
case_images: Response on pazopanib treatment.
case_images: fonc-11-641376-g0003.jpg
case_images: PMC8032946_01_fonc-11-641376-g0003.jpg
case_images: F3
case_images: With the combination of pazopanib (800 mg 1x/day po) and denosumab, there was regression of the subcutaneous disease and stability of lung and bone metastasis for over a year Figure 3.