A case report of epithelioid renal angiomyolipoma with inferior vena cava extension: robotic surgical management and literature review of rare presentation

Abstract

BACKGROUND: Epithelioid angiomyolipoma (EAML) is an uncommon renal tumor variant with histologic and radiologic features that can mimic renal cell carcinoma (RCC) on imaging due to the paucity of fat compared to the classic AML. EAML may exhibit aggressive behavior, including local invasion, recurrence, and distant metastases to the liver, lungs, and lymph nodes. Although recent reports suggest that up to one-third of EAML cases may behave malignantly, variability in diagnostic criteria and limited case series contribute to uncertainty regarding its true clinical course. CASE DESCRIPTION: This case report describes a 19-year-old female presenting with an 11.9 cm right renal EAML with a tumor thrombus extending into the inferior vena cava (IVC). She underwent a robotic radical nephrectomy with IVC thrombectomy and lymph node dissection. The final pathology revealed EAML with negative margins. Follow-up computed tomography (CT) imaging demonstrated no recurrence of disease/metastasis. The rarity of this presentation and the lack of consensus on management highlight the importance of reporting such cases. CONCLUSIONS: This rare case of EAML with IVC tumor thrombus demonstrates that timely robotic surgical intervention with appropriate follow-up is critical for the appropriate management of renal EAML.