Giant renal pseudoaneurysm complicating angiomyolipoma in a patient with tuberous sclerosis complex: An unusual case report and review of the literature.

INTRODUCTION

Angiomyolipomas (AMLs) are uncommon benign lesions, which are composed of dysmorphic blood vessels, adipose tissue, and smooth muscle components. They tend to bleed because of the hypervascularity and the presence of small aneurysms, leading to life-threatening complications.

PRESENTATION OF CASE

A 31-year-old female was presented to the emergency service of our hospital, complaining of left flank pain for 1 week followed by hematuria for one day. Radiologic imaging showed the features of a giant renal pseudoaneurysm. Superselective embolization was applied and she had an uneventful recovery.

DISCUSSION

The blood vessels in AML are tortuous and thick-walled with the absence of supportive elastic tissue, which tend to the formation of the intralesional pseudoaneurysm. The risk of bleeding is higher with tumors larger than 4 cm, rapid tumor growth, and aneurysms larger than 0.5 cm. Early detection and treatment are essential for the prevention of bleeding and improving patient outcomes.

CONCLUSION

Giant pseudoaneurysm in a renal angiomyolipoma associated with tuberous sclerosis complex is a rare entity, often leading to potentially life-threatening bleeding. Selective angioembolization is recommended as firstline therapy for bleeding AML and is increasingly used as a preventive treatment for AML at risk of bleeding. However, a high incidence of the recurrence requires caution and a close longtime follow-up. Surgical intervention is indicated if the hemorrhage is not responsive to embolization or if there is suspicion of malignancy.