Angiomiolipoma hemorrágico espontáneo asociado a esclerosis tuberosa que conduce a nefrectomía urgente – Reporte de un caso y revisión de la literatura

Description: A 34-year-old woman came to the emergency department presenting with moderate abdominal pain in the right hypochondrium (of 3-hour duration), accompanied by hematuria and progressive and painful abdominal distention. A tomography scan revealed a 14 cm heterogenous solid tumor in the right kidney, with intratumoral bleeding. She was given a blood product transfusion due to low hemodynamic output and kept under surveillance. Hemoglobin levels continued to descend, reaching 7.2 g/dl, and so the patient underwent emergency selective angioembolization, and the bleeding temporarily stopped. The hematuria reappeared and the patient went into hypovolemic shock, leading to the definitive surgical treatment of total nephrectomy. Her recovery was uneventful. The histopathologic study reported renal angiomyolipoma with extensive bleeding. Relevance: Wunderlich syndrome (angiomyolipoma with spontaneous bleeding) is a rare entity whose most severe and undesirable complication is refractory bleeding, which can be fatal. Clinical implications: Early intervention in patients with hemorrhagic angiomyolipoma is important for preventing potentially life-threatening complications, such as massive bleeding. Conclusions: In cases of angiomyolipoma with intratumoral bleeding and symptoms of Wunderlich syndrome, total nephrectomy is the best option, after conservative management and/or selective angioembolization, for saving the life of the patient.