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Kidney Week

Abstract: SA-PO217

Challenges in the Management of Giant Bilateral Renal Angiomyolipomas in a Young Woman with Tuberous Sclerosis Complex: A Case Report

Session Information

Category: Onconephrology

  • 1700 Onconephrology

Author

  • Dinsay, Mark Gavin Coronel, Philippine General Hospital, Manila, Metro Manila, Philippines
Introduction

Tuberous sclerosis complex-associated renal angiomyolipoma (AML) is a fast-growing tumor that develops earlier in life, with a propensity to achieve larger sizes and higher risks for life-threatening hemorrhage. We report a rare case of bilateral giant AMLs wherein a young patient presented with extensive involvement in both kidneys that limited treatment options recommended by current guidelines.

Case Description

A 23-year-old woman with tuberous sclerosis complex (TSC) presented with flank pain, abdominal fullness, hematuria, and hemodynamic instability. CT scan of the abdomen showed that both kidneys had been converted into large aggregates of heterogeneously enhancing and attenuating masses. The right kidney measured 17.4 x 9.7 x 10.3 cm (CC x W x AP) while the left measured 18.4 x 11.4 x 16.2 cm (CC x W x AP). She was transfused 2 units of packed red blood cells and closely monitored. Radiologic intervention with angioembolization was deemed unfeasible due to the diffuse involvement of both kidneys. Bleeding resolved spontaneously albeit with a high risk for recurrence. Prophylactic bilateral nephrectomy was considered but would render the patient in need of renal replacement therapies. After a multidisciplinary conference and discussion with the patient and her family, conservative management and therapy with mTOR inhibitor everolimus were pursued. The patient was apprised of the need for nephrectomy should refractory life-threatening bleeding occur. On outpatient follow-up after a year, renal function was preserved with an eGFR of 87 mL/min. Although everolimus was discontinued after 1 month due to financial constraints, the patient reported subjective improvement in symptoms and no recurrence of anemia and bleeding.

Discussion

In our patient's case, extensive involvement of both kidneys and massive sizes of the angiomyolipoma presented a dilemma that required balancing the patient’s preferences, risk for bleeding, and preservation of renal function. A conservative management approach involves close monitoring, intensive care, and blood transfusions. mTOR inhibition is employed to retard tumor size and growth and reduce the risk for bleeding. The challenge with managing such cases requires a multidisciplinary approach and shared decision-making to arrive at the best patient-centered outcomes.