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Case Reports
. 2022 Apr 5;14(4):e23861.
doi: 10.7759/cureus.23861. eCollection 2022 Apr.

Wunderlich Syndrome Associated With Angiomyolipomas

Affiliations
Case Reports

Wunderlich Syndrome Associated With Angiomyolipomas

David Antonio Ramirez-Limon et al. Cureus. .

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.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
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
Figure 2. Macroscopic illustration of the nephrectomy specimen. (A) anterior and (B) posterior view.
PESO: Weight
Figure 3
Figure 3. Sagittal cut of the specimen with subcapsular hematoma pointed by the black arrows.
Figure 4
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
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
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
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).

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References

    1. Wunderlich syndrome. Chen YC, Lin YC. QJM. 2013;106:187–188. - PubMed
    1. The Herlyn-Werner-Wunderlich (HWW) syndrome - a case report with radiological review. Hayat AM, Yousaf KR, Chaudhary S, Amjad S. Radiol Case Rep. 2022;17:1435–1439. - PMC - PubMed
    1. Wunderlich syndrome, spontaneous ruptured renal angiomyolipoma and tuberous sclerosis. Ho TH, Yang FC, Cheng KC, Lin CC, Lee JT. QJM. 2019;112:283–284. - PubMed
    1. Wunderlich syndrome: cross-sectional imaging review. Katabathina VS, Katre R, Prasad SR, Surabhi VR, Shanbhogue AK, Sunnapwar A. J Comput Assist Tomogr. 2011;35:425–433. - PubMed
    1. Spontaneous perirenal hemorrhage (Wunderlich syndrome): an analysis of 28 cases. Kim JW, Jung YK, Ahn ST, et al. Am J Emerg Med. 2019;37:45–47. - PubMed

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