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Renal angioleiomyoma is an exceptionally rare benign mesenchymal tumor, with fewer than five cases documented in the literature.Histologically, it is composed of proliferating smooth muscle cells intersected by branching, slit-like vascular channels and typically lacks significant epithelial components.Despite its benign nature, its morphological overlap with certain renal malignancies presents a diagnostic challenge.Notably, renal cell carcinoma with angioleiomyoma-like stroma, a recently recognized variant of renal cell carcinoma, demonstrates a histologically and immunohistochemically similar stromal component.Although it generally exhibits a more indolent clinical course, there have been reports of lymph node involvement, underscoring its malignant potential.In this report, we present a case of renal angioleiomyoma and discuss the importance of distinguishing it from its malignant counterpart.Histopathological evaluation, supplemented by immunohistochemistry, plays a vital role in achieving a definitive diagnosis.Accurate differentiation is crucial to avoid overtreatment and to ensure appropriate clinical management.The overall prognosis is excellent, reflecting the inherent benign nature of renal angioleiomyoma.No routine long-term surveillance is required once a diagnosis is accurately confirmed.However, pathological overlap with renal cell carcinoma with angioleiomyoma-like stroma may necessitate selective follow-up in cases of diagnostic ambiguity.Improved awareness of renal angioleiomyoma helps ensure correct diagnosis and prevents confusion with malignant or other benign mesenchymal tumors that involve the kidney.
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Shah et al. (2026) studied this question.
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