Robotic Treatment of Adrenal Sclerosing PEComa: A Case Report with 13 Years of Follow-Up and a Literature Review

Background: Sclerosing perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, typically benign but occasionally exhibiting aggressive behavior. This study reports a case of sclerosing PEComa of the adrenal gland that was treated with robotic partial adrenalectomy, with 13 years of follow-up and a review of the literature. Methods: A 48-year-old woman presented with abdominal pain. Imaging revealed a 10 × 9 cm adrenal mass displacing adjacent structures. MRI and 18F-FDG PET-CT suggested angiomyolipoma. Robotic partial adrenalectomy was performed. Intraoperative frozen section analysis identified the mass as angiomyolipoma, while final histopathology confirmed it as a sclerosing PEComa. Results: This case highlights the advantages of robotic surgery in the management of large adrenal tumors, allowing complete tumor removal while preserving functional adrenal tissue. The extended 13-year follow-up is significant, given the potential for recurrence or malignant transformation reported in other cases. A literature review identified 39 reported cases of sclerosing PEComas, with few documenting long-term outcomes. Conclusions: This is a documented case of robotic partial adrenalectomy for sclerosing PEComa. The findings support robotic surgery as a safe effective approach for managing this rare tumor, with excellent functional and oncological results. Extended follow-up reinforces the tumor’s benign behavior and the importance of long-term monitoring in PEComas.