Partial Nephrectomy for a Massive Sporadic Renal Angiomyolipoma: Case Report and Review of the Literature.

Jacob Albersheim-Carter,Molly Klein,Paari Murugan,Christopher J. Weight

doi:10.1155/2016/3420741

Jacob Albersheim-Carter, Molly Klein + Show 2 more

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https://doi.org/10.1155/2016/3420741

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Abstract

Introduction. Angiomyolipomas are the most common benign tumor of the kidney, associated with Tuberous Sclerosis in 20% of cases and arising sporadically in 80% of cases. Renal angiomyolipomas are neoplasms of mesenchymal origin with varying proportions of vasculature, smooth muscle spindle cells, and adipocytes, making management of such neoplasms a challenging endeavor. Possible management options include partial or radical nephrectomy and segmental renal artery embolization. Case Presentation. A 61-year-old woman admitted for a large retroperitoneal hemorrhage was discovered to have a giant, sporadic, 3818.3 g, 30.0 × 26.5 × 18.0 cm left perinephric angiomyolipoma. Given her hemodynamic instability upon presentation, she underwent segmental arterial embolization, followed by an open left partial nephrectomy. Ten-month follow-up revealed no noticeable loss of renal function. Discussion. Literature review revealed occasional renal angiomyolipomas of comparable size, with all angiomyolipomas larger than this requiring treatment with radical nephrectomy. Conclusion. We show that nephron-sparing surgery may be considered in the treatment of even the largest of renal angiomyolipomas.

Highlights

Angiomyolipomas are the most common benign tumor of the kidney, associated with Tuberous Sclerosis in 20% of cases and arising sporadically in 80% of cases
We report a clinical case of symptomatic giant sporadic AML in a 61-year-old woman treated with segmental arterial embolization (SAE) followed closely by partial nephrectomy with maintenance of normal renal function
She underwent an open exploration of her left renal AML, with plan for intraoperative evaluation and consideration of a partial versus radical nephrectomy based on the extent of bleeding and inflammation in the retroperitoneal space

Summary

Introduction

Angiomyolipomas (AMLs), a type of perivascular epithelioid cell tumor (PEComa), are the most common benign tumor of the kidney, yet are relatively uncommon in the general population, occurring in 0.3%–2.1% of people. Women are more frequently affected than men, with a female-to-male ratio of 11 : 4 [1]. Renal AMLs may arise sporadically or as a consequence of tuberous sclerosis complex (TSC). AMLs arising sporadically have displayed different characteristics compared to AMLs associated with TSC. TSC AMLs have exhibited larger average sizes, more frequent multiple and bilateral lesions, faster growth rate, and earlier presentation compared to sporadic AMLs [2]. Intervention for symptomatic AMLs has included radical nephrectomy or partial nephrectomy, with or without arterial embolization, and embolization alone [3]. We report a clinical case of symptomatic giant sporadic AML in a 61-year-old woman treated with segmental arterial embolization (SAE) followed closely by partial nephrectomy with maintenance of normal renal function

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Discussion