No abstract

woman had FIGO-IIb PEComa with recurrence within 2 months following surgery in vagina and lung. She underwent vaginal and thoracic surgery resecting tumors. She did not receive chemotherapy for ovarian cancer due to medical fitness. PEComa relapsed aggressively in the pelvis, lungs and diaphragm within 6 months and she was treated with Sirolimus and Nab-sirolimus. With this aggressive tumour, her overall survival was 14 months. Conclusion This illustrates the natural history of a rare uterine tumour (PEComa) and the management in rare presentations with aggressive tumours. The management can be challenging requiring mutidisciplinary approach. There is lack of evidence how to manage recurrence of PEComa not salvaged by surgery.

No abstract