Literature DB >> 26435953

Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich's Syndrome in A Tuberous Sclerosis Patient.

Sushma Shankar1, Kiran Shankar Hulikanthimatt2, Shreeharsha Mallappa Awatti3, Suresh Turuvekere Narayanrao4.   

Abstract

Renal Angiomyolipoma (renal AML) is a benign clonal neoplasm with a incidence of 0.3-3%, occurring as isolated sporadic entity or in association with Tuberous sclerosis (TS) in 80% cases. Multiple, bilateral renal AML are considered diagnostic of Tuberous sclerosis. Wunderlich's syndrome, a urological emergency is a spontaneous nontraumatic bleeding into subcapsular and or peri-renal space and is a life threatening complication of renal AML occurring in 10% cases which has to be timely diagnosed and treated. Here, we present an unusual case of TS with bilateral, multifocal renal AML in a 25-year-old female who presented with Wunderlich's syndrome, which is a rare but life threatening complication that has to be suspected, timely diagnosed and treated. We have discussed the importance of early diagnosis, timely treatment, follow up and education of patient and relatives of the possible complications associated.

Entities:  

Keywords:  Bilateral; Female; Life threatening

Year:  2015        PMID: 26435953      PMCID: PMC4576544          DOI: 10.7860/JCDR/2015/13349.6289

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  13 in total

1.  Phenotypes of the tuberous sclerosis complex with a revision of diagnostic criteria.

Authors:  M R Gomez
Journal:  Ann N Y Acad Sci       Date:  1991       Impact factor: 5.691

2.  Concurrent bilateral renal angiomyolipoma and renal cell carcinoma in a patient with tuberous sclerosis complex.

Authors:  Abdelhak Khallouk; Younes Ahallal; Mbarek Doublali; Mohamed Fadl Tazi; Soufiane Mellas; Mohamed Jamal El Fassi; Moulay Hassan Farih
Journal:  Rev Urol       Date:  2009

3.  Giant renal angiomyolipomas and pulmonary lymphangiomyomatosis.

Authors:  Kiran Nasir; Aasim Ahmad
Journal:  Saudi J Kidney Dis Transpl       Date:  2010-03

4.  Wunderlich's Syndrome in a Tuberous Sclerosis Patient.

Authors:  Surya Prakash Vaddi; Vedamurthy Pogula Reddy; Rahul Devraj
Journal:  Indian J Surg       Date:  2011-01-27       Impact factor: 0.656

5.  Wunderlich's syndrome with hepatic angiomyolipoma in tuberous sclerosis.

Authors:  R Mongha; P Bansal; A Dutta; R K Das; A K Kundu
Journal:  Indian J Cancer       Date:  2008 Apr-Jun       Impact factor: 1.224

6.  Tuberous sclerosis with bilateral renal angiomyolipoma and wunderlich's syndrome.

Authors:  Noor Nabi; Aatif Hassan Shaikh; Salman-El Khalid
Journal:  J Coll Physicians Surg Pak       Date:  2007-11       Impact factor: 0.711

7.  Malignant transformation of renal angiomyolipoma.

Authors:  Nobuyoshi Takahashi; Ryuji Kitahara; Yasuyuki Hishimoto; Akimitsu Ohguro; Yasuhiro Hashimoto; Tadashi Suzuki
Journal:  Int J Urol       Date:  2003-05       Impact factor: 3.369

8.  Wunderlich's syndrome in spontaneous angiomyolipoma bleeding.

Authors:  G H Neild; Chun-Yu Lin; Yih-Huei Lin; Fu-Zong Wu; Huay-Ben Pan
Journal:  NDT Plus       Date:  2011-03-01

9.  Wunderlich's syndrome and hemorrhagic shock.

Authors:  Massimo Medda; Stefano Cm Picozzi; Giorgio Bozzini; Luca Carmignani
Journal:  J Emerg Trauma Shock       Date:  2009-09

10.  Spontaneous Retroperitoneal Hemorrhage (Wunderlich Syndrome) due to Large Upper Pole Renal Angiomyolipoma: Does Robotic-Assisted Laparoscopic Partial Nephrectomy Have a Role in Primary Treatment?

Authors:  Achilles Ploumidis; Ioannis Katafigiotis; Maria Thanou; Nikos Bodozoglou; Labros Athanasiou; Antonios Ploumidis
Journal:  Case Rep Urol       Date:  2013-09-11
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  1 in total

1.  Wunderlich's Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma.

Authors:  Sara Catarino Santos; Liliana Duarte; Fernando Valério; Júlio Constantino; Jorge Pereira; Carlos Casimiro
Journal:  Am J Case Rep       Date:  2017-12-08
  1 in total

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