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Abstract
Introduction The perivascular epithelioid cell neoplasm is a group of mesenchymal tumor originating from the perivascular epithelioid cell line that may affect different organs, but sharing distinctive morphologic and immunohistochemical features. Our objective is to present the retroperitoneal extended resection for para-aortic isolated late recurrence of a rare tumor.
Methods A case report written based on the retrospective review of the medical records of a patient diagnosed with PEComa.
Results Case report of a 46 years old patient diagnosed initially with a low grade uterine sarcoma after a hysterectomy for myomatosis. After 8 years she presented with back pain and the MRI diagnosed a retroperitoneal mass involving the aorta 145 degrees, from below the renal hilum up to the left common iliac artery, with 96 × 66 × 74 mm. The PET-CT showed no distant metastasis. The patient was submitted to a resection of the retroperitoneal mass, left nephrectomy, with aorta resection, followed by vascular reconstruction with aorto-bi-iliac prosthesis. The histological findings showed an uterine PEComa, positive for HMB-45, Melan-A, CALP, D33 and 1A4. The patient was then submitted to adjuvant chemotherapy with gemcitabine and docetaxel with radiotherapy. After 11 months of follow-up, she had no retroperitoneal or pelvic recurrence, but were diagnosed pulmonar and bone metastasis. She is now in treatment with rapamycin and denosumab.
Conclusion Extended retroperitoneal resection with vascular reconstruction may be an option for local control in selected isolated late recurrence of slow growing gynecologic tumors.