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Organo Oficial de la Sociedad Mexicana de Urología

2020, Number 5

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Rev Mex Urol 2020; 80 (5)

Angioembolization prior to nephrectomy in Wunderlich syndrome: a case report of collision angiomyolipoma and clear cell renal cell carcinoma

Pallares-Méndez R, Cervantes-Miranda DE, Hernández-Aranda KL, Gutiérrez-González A, Arrambide-Gutiérrez G

Language: English
References: 22
Page: 1-8
PDF size: 233.47 Kb.


ABSTRACT

A 29-year-old man with previously undiagnosed tuberous sclerosis complex arrived at the emergency department due to anemia-related gross hematuria. A computed tomography scan revealed a bilateral renal mass consistent with angiomyolipoma and a right retroperitoneal hematoma. Angioembolization of the right renal artery was performed prior to nephrectomy. The histopathologic results were collision angiomyolipoma and clear cell renal cell carcinoma.
Relevance: The multisystemic presentation of tuberous sclerosis complex combined with the histologic findings of collision angiomyolipoma and clear cell renal cell carcinoma is a rare entity in the medical literature.
Clinical implications: Angioembolization results in fewer postoperative complications of ileus, pain, or infection, and its utility in the present case was important for facilitating the approach during nephrectomy and reducing the risk for intraoperative bleeding.
Conclusions: The present case report provides a description of Wunderlich syndrome in a patient with tuberous sclerosis complex and the histopathologic diagnosis of a collision tumor, consisting of angiomyolipoma and clear cell renal cell carcinoma. Angioembolization of the right renal artery was performed before nephrectomy, reducing the risk for intraoperative bleeding.


REFERENCES

  1. Chamarthi G, Koratala A. Wunderlich syndrome. Clinical Case Reports. 2018;6(9):1901–2. doi: https://doi.org/10.1002/ccr3.1738

  2. Albi G, Campo L del, Tagarro D. Wünderlich’s Syndrome: Causes, Diagnosis and Radiological Management. Clinical Radiology. 2002 Sep 1;57(9):840–5. doi: https://doi.org/10.1053/ crad.2002.0981

  3. O’Callaghan FJ, Shiell AW, Osborne JP, Martyn CN. Prevalence of tuberous sclerosis estimated by capture-recapture analysis. The Lancet. 1998 May 16;351(9114):1490. doi: https://doi. org/10.1016/S0140-6736(05)78872-3

  4. Leung AKC, Robson WLM. Tuberous Sclerosis Complex: A Review. Journal of Pediatric Health Care. 2007 Mar 1;21(2):108–14. doi: https:// doi.org/10.1016/j.pedhc.2006.05.004

  5. Kwiatkowski DJ, Manning BD. Molecular basis of giant cells in tuberous sclerosis complex. N Engl J Med. 2014 Aug 21;371(8):778–80. doi: https://doi.org/10.1056/nejmcibr1406613

  6. Northrup H, Krueger DA, Northrup H, Krueger DA, Roberds S, Smith K, et al. Tuberous Sclerosis Complex Diagnostic Criteria Update: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatric Neurology. 2013 Oct 1;49(4):243–54. doi: https://doi.org/10.1016/j. pediatrneurol.2013.08.001

  7. Chu-Shore CJ, Major P, Camposano S, Muzykewicz D, Thiele EA. The natural history of epilepsy in tuberous sclerosis complex. Epilepsia. 2010;51(7):1236–41. doi: https://doi. org/10.1111/j.1528-1167.2009.02474.x

  8. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal Angiomyolipoma: Relationships between Tumor Size, Aneurysm Formation, and Rupture. Radiology. 2002 Oct 1;225(1):78–82. doi: https://doi.org/10.1148/radiol.2251011477

  9. Santos SC, Duarte L, Valério F, Constantino J, Pereira J, Casimiro C. Wunderlich’s Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma. Am J Case Rep. 2017 Dec 8;18:1309–14. doi: https://doi. org/10.12659/ajcr.905975

  10. Sun P, Liu J, Charles H, Hulbert J, Bissler J. Outcomes of angioembolization and nephrectomy for renal angiomyolipoma associated with tuberous sclerosis complex: a real-world US national study. Current Medical Research and Opinion. 2017 May 4;33(5):821– 7. doi: https://doi.org/10.1080/03007995.2017 .1286307

  11. Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg BA, Frost M, Belousova E, et al. Everolimus for renal angiomyolipoma in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis: extension of a randomized controlled trial. Nephrol Dial Transplant. 2016 Jan 1;31(1):111–9. doi: https://doi.org/10.1093/ndt/gfv249

  12. Chamarthi G, Koratala A. Wunderlich syndrome. Clinical Case Reports. 2018;6(9):1901–2. doi: https://doi.org/10.1002/ccr3.1738

  13. Albi G, Campo L del, Tagarro D. Wünderlich’s Syndrome: Causes, Diagnosis and Radiological Management. Clinical Radiology. 2002 Sep 1;57(9):840–5. doi: https://doi.org/10.1053/ crad.2002.0981

  14. O’Callaghan FJ, Shiell AW, Osborne JP, Martyn CN. Prevalence of tuberous sclerosis estimated by capture-recapture analysis. The Lancet. 1998 May 16;351(9114):1490. doi: https://doi. org/10.1016/S0140-6736(05)78872-3

  15. Leung AKC, Robson WLM. Tuberous Sclerosis Complex: A Review. Journal of Pediatric Health Care. 2007 Mar 1;21(2):108–14. doi: https:// doi.org/10.1016/j.pedhc.2006.05.004

  16. Kwiatkowski DJ, Manning BD. Molecular basis of giant cells in tuberous sclerosis complex. N Engl J Med. 2014 Aug 21;371(8):778–80. doi: https://doi.org/10.1056/nejmcibr1406613

  17. Northrup H, Krueger DA, Northrup H, Krueger DA, Roberds S, Smith K, et al. Tuberous Sclerosis Complex Diagnostic Criteria Update: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatric Neurology. 2013 Oct 1;49(4):243–54. doi: https://doi.org/10.1016/j. pediatrneurol.2013.08.001

  18. Chu-Shore CJ, Major P, Camposano S, Muzykewicz D, Thiele EA. The natural history of epilepsy in tuberous sclerosis complex. Epilepsia. 2010;51(7):1236–41. doi: https://doi. org/10.1111/j.1528-1167.2009.02474.x

  19. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal Angiomyolipoma: Relationships between Tumor Size, Aneurysm Formation, and Rupture. Radiology. 2002 Oct 1;225(1):78–82. doi: https://doi.org/10.1148/radiol.2251011477

  20. Santos SC, Duarte L, Valério F, Constantino J, Pereira J, Casimiro C. Wunderlich’s Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma. Am J Case Rep. 2017 Dec 8;18:1309–14. doi: https://doi. org/10.12659/ajcr.905975

  21. Sun P, Liu J, Charles H, Hulbert J, Bissler J. Outcomes of angioembolization and nephrectomy for renal angiomyolipoma associated with tuberous sclerosis complex: a real-world US national study. Current Medical Research and Opinion. 2017 May 4;33(5):821– 7. doi: https://doi.org/10.1080/03007995.2017 .1286307

  22. Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg BA, Frost M, Belousova E, et al. Everolimus for renal angiomyolipoma in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis: extension of a randomized controlled trial. Nephrol Dial Transplant. 2016 Jan 1;31(1):111–9. doi: https://doi.org/10.1093/ndt/gfv249




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