Wunderlich Syndrome Associated With Angiomyolipomas

Abstract

Wünderlich syndrome (WS) is a spontaneous retroperitoneal hemorrhage confined to the subcapsular or perinephric space without a history of trauma. Since it is a rare condition with a significant mortality rate if not treated timely, it is essential to identify its risk factors and early clinical manifestations for a favorable outcome. Various conditions are associated, but the most common causes are benign and malignant renal neoplasms. We present a 26-year-old female with a history of tonic-clonic seizures who presented to the ED with intense abdominal pain located on the right flank with a palpable mass. Management included IV fluids and blood transfusion. She underwent a right total nephrectomy. She was later diagnosed with tuberous sclerosis. A 44-year-old female with a three-year history of right costovertebral pain and recurrent urinary tract infections that presented to the ED with acute right flank pain was diagnosed with WS secondary to an angiomyolipoma and underwent right total nephrectomy. WS is a very rare pathology that represents a diagnostic challenge for the physician. The treatment will depend on the hemodynamic condition of the patient. Active follow-up should be reserved for those who have small tumors, are asymptomatic, and have hemodynamic stability. Surgical or radiology intervention is reserved for those who are hemodynamically unstable or who have a suspicion of renal cell carcinoma.

Keywords: flank pain; herlyn-werner-wunderlich syndrome; lenk's triad; perirenal hematoma; renal angiomyolipoma.

Figure 1. Female patient with small angiofibromas in her face (A,B) and ash leaf marks in her back (C) and lower extremity (D).

Figure 2. Macroscopic illustration of the nephrectomy specimen. (A) anterior and (B) posterior view.

PESO: Weight

Figure 3. Sagittal cut of the specimen with subcapsular hematoma pointed by the black arrows.

Figure 4. Microscopic slide of the inferior pole of the kidney with hematoxylin and eosin stain showing fat (black arrow) and smooth muscle spindle shaped (red arrow) components

Figure 5. Angiomyolipoma with hematoxylin and eosin stain. Classic variant composed of thick walled sclerosed vessels (black arrows), myoid epithelioid cells (red arrows) and fat (yellow arrow).

Figure 6. CT scan with axial view showing a heterogenous mass (white arrows) shifting the renal parenchyma to the right with multiple components.

Figure 7. CT scan with coronal view showing the renal parenchyma shifted to the right and a mass with hypo and hyperdense components within the renal capsule (white arrows).