Introduction: Herein we present a rare case of pontaneous rupture of a giant renal angiomyolipoma (AML), with symptoms of hypovolemic shock (Wunderlich's syndrome), which was managed by urgent total nephrectomy. Presentation of case: A 53 year old female was transferred to the emergency room with progressive acute painful swelling of the left lateral abdominal area, duration of 5h. An emergent ultrasonic examination, revealed a heterogeneous-solid mass with maximum diameter of 23cm, with probable origin from the left kidney. Due to worsening of the clinical status (hypovolemic shock), loss of consciousness and acute drop of haematocrit level to 17.8%, the patient was urgently intubated in the emergency room and transferred to the operating theater. A giant haemorrhagic mass was found originating from the left kidney, which removed en-block with the left kidney. The patient was transferred to the intensive care unit. Her recovery was uneventful. The histopathologic examination revealed a giant renal angiomyolipoma (25x18x8cm) with extensive bleeding. Discussion: Enlarged renal AMLs can rupture. This can be sudden and painful with manifestations of hypovolemic shock. The management of AMLs has been correlated with symptoms. Patients with life-threatening retroperitoneal haemorrhage, require urgent exploration as retroperitoneal bleeding can lead to severe complications, increasing morbidity. Conclusion: In case of giant angiomyolipoma with intratumoral haemorrhage, and symptoms of Wunderlich's syndrome, partial or total nephrectomy is a good treatment option in order to save the patient's life.
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