Tuberoskleroz Kompleksli Hastada Hemorajik Giant Renal Anjiomyolipom a Nefron Koruyucu Cerrahi Yaklaşım: Olgu Sunumu
Öz
Tuberoskleroz, tüm organları tutabilen ancak en sık deri, beyin, kalp, göz ve böbreği tutan benign hamartomatöz lezyonlar ile karakterize otozomal dominant bir hastalıktır. Renal anjiomyolipom AML matür yağ hücreleri, düz kas dokusu ve kalın duvarlı kan damarlarını değişen oranlarda içeren benign bir tümördür. Tuberoskleroz kompleksinin TS en yaygın ürolojik tutulumu renal anjiomyolipomlardır. Anjiomyolipomlar asemptomatik olabilmekle beraber, palpabl kitle, ağrı, hematüri şikayeti ile tanı alabilir, masif kanamaya neden olarak morbidite ve mortaliteye sebep olabilirler. Biz de palpable kitle ile tanı konulan, masif kanamaya neden olan ve cerrahi tedavi yapılan dev renal anjiomyolipom olgumuzu paylaşmak ve bulgular eşliğinde güncel literatürü değerlendirmek istedik
Anahtar Kelimeler
Kaynakça
- Winterkorn EB, Daouk GH, Anupindi S, et al. Tuberous sc- lerosis complex and renal angiomyolipoma: case report and review of the literature. Pediatr Nephrol 2006; 21: 1189-93.
- Kılıç Ö, Yurdakul T, Kaynar M, Özbek O, Baba F. Tübe- rosklerozlu hastada bilateral renal anjiomyolipom sebebi ile masif retroperitonael kanama. Gazi Medical Journal 2009;20:86-9.
- Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA. Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors. Kid- ney Int 2006;70:1777-82.
- Ünlü EN, Atasoy Ç, Fitöz S, Yağcı C, Akyar S. Tüberosk- lerozda dev renal anjiyomyolipomlar:Bilgisayarlı tomog- rafi bulguları. Ankara Üniversitesi Tıp Fakültesi Mecmuası 2005;58:189-92.
- Kontos S, Politis V, Fokitis I, et al. Rupture of renal angiom- yolipoma during pregnancy: a case report, Cases Journal 2008;1:245.
- Schillinger F, Montagnac R. Renal lesions in tuberous scle- rosis. Nephrol Ther 2006;2 Suppl 2:S123-6.
- Kushwaha R, Dhawan I, Arora R, Gupta K, Dhupia JS. Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage. Indian J Pathol Microbiol 2010;53:340-1.
- Park HK, Zhang S, Wong MK, Kim HL. Clinical presentati- on of epithelioid angiomyolipoma, Int J Urol 2007; 14:21-5.
- Orlova KA, Crino PB. The tuberous sclerosis complex. Ann NY Acad Sci 2010;1184:87-105.
- Mistry KA, Sood D, Bhoil R, et al. A Classic Case of Tu- berous Sclerosis with Multisystem Involvement Including Giant Bilateral Renal Angiomyolipomas Presenting as Mas- sive Hematuria. Pol J Radiol 2015;80:435-41.
- Hallett L, Foster T, Liu Z, et al. Burden of disease and un- met needs in tuberous sclerosis complex with neurologi- cal manifestations: systematic review. Curr Med Res Opin 2011;27:1571-83.
- Shankar S, Hulikanthimatt KS, Awatti SM, Narayanrao ST. Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich’s Syndrome in A Tuberous Sclerosis Pa- tient. J Clin Diagn Res 2015; 9:ED01-3.
- Taken M, Pirinççi N, Kaba1 M, Ergün M, Canbey Ö. Re- nal Anjiomyolipomdan Dolayı Massif Retroperitoneal Kanama: Bir Olgu Sunumu. Tıp Araştırmaları Dergisi 2015;13:83-5.
- Roach ES, Gomez MR. Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13:624-8.
- Kapoor A, Girard L, Lattouf JB, et al. Evolving Strategies in the Treatment of Tuberous Sclerosis Complex-Associated Angiomyolipomas (TSC-AML). Urology 2016;89:19-26.
- Mullerad M, Hidas G, Kastin A, Issaq E, Moskovitz B, Nativ O. Nephron sparing surgery as a treatment modality for re- nal angiomyolipoma. Harefuah 2005;144:619-21.
- Krueger DA, Northrup H. Tuberous Sclerosis Complex Surveillance and Management: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013;49:255-65.
- Yılmaz E, Özdemir K, Başaran C, Gözmen ŞK, Erturgut P, Serdaroğlu E. Kidney involvement in tuberous sclerosis complex. Dicle Med J 2015;42:66-71.
Öz
Tuberous sclerosis is an autosomal dominant disease characterized with benign hamartomatosis lesions which may involve all organs but most commonly involve skin, brain, heart, eye and kidney. Renal angiomyolipoma AML is a benign tumor including mature fat cells, flat muscle tissue and thick walled blood vessels in different ratios. Angiomyolipomas are the most common urological involvement of tuberous sclerosis complex TS . Although angiomyolipomas may be asymptomatic, they can be diagnosed with palpable mass, pain and hematuria complaint and may cause morbidity and mortality by massive bleeding. We wanted to share our giant renal angiomyolipoma case which was diagnosed with a palpable mass and caused a massive bleeding and was treated surgically and review the current literature with the findings
Anahtar Kelimeler
Renal angiomyolipoma Tuberous Sclerosis Nephron sparing surgery
Kaynakça
- Winterkorn EB, Daouk GH, Anupindi S, et al. Tuberous sc- lerosis complex and renal angiomyolipoma: case report and review of the literature. Pediatr Nephrol 2006; 21: 1189-93.
- Kılıç Ö, Yurdakul T, Kaynar M, Özbek O, Baba F. Tübe- rosklerozlu hastada bilateral renal anjiomyolipom sebebi ile masif retroperitonael kanama. Gazi Medical Journal 2009;20:86-9.
- Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA. Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors. Kid- ney Int 2006;70:1777-82.
- Ünlü EN, Atasoy Ç, Fitöz S, Yağcı C, Akyar S. Tüberosk- lerozda dev renal anjiyomyolipomlar:Bilgisayarlı tomog- rafi bulguları. Ankara Üniversitesi Tıp Fakültesi Mecmuası 2005;58:189-92.
- Kontos S, Politis V, Fokitis I, et al. Rupture of renal angiom- yolipoma during pregnancy: a case report, Cases Journal 2008;1:245.
- Schillinger F, Montagnac R. Renal lesions in tuberous scle- rosis. Nephrol Ther 2006;2 Suppl 2:S123-6.
- Kushwaha R, Dhawan I, Arora R, Gupta K, Dhupia JS. Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage. Indian J Pathol Microbiol 2010;53:340-1.
- Park HK, Zhang S, Wong MK, Kim HL. Clinical presentati- on of epithelioid angiomyolipoma, Int J Urol 2007; 14:21-5.
- Orlova KA, Crino PB. The tuberous sclerosis complex. Ann NY Acad Sci 2010;1184:87-105.
- Mistry KA, Sood D, Bhoil R, et al. A Classic Case of Tu- berous Sclerosis with Multisystem Involvement Including Giant Bilateral Renal Angiomyolipomas Presenting as Mas- sive Hematuria. Pol J Radiol 2015;80:435-41.
- Hallett L, Foster T, Liu Z, et al. Burden of disease and un- met needs in tuberous sclerosis complex with neurologi- cal manifestations: systematic review. Curr Med Res Opin 2011;27:1571-83.
- Shankar S, Hulikanthimatt KS, Awatti SM, Narayanrao ST. Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich’s Syndrome in A Tuberous Sclerosis Pa- tient. J Clin Diagn Res 2015; 9:ED01-3.
- Taken M, Pirinççi N, Kaba1 M, Ergün M, Canbey Ö. Re- nal Anjiomyolipomdan Dolayı Massif Retroperitoneal Kanama: Bir Olgu Sunumu. Tıp Araştırmaları Dergisi 2015;13:83-5.
- Roach ES, Gomez MR. Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13:624-8.
- Kapoor A, Girard L, Lattouf JB, et al. Evolving Strategies in the Treatment of Tuberous Sclerosis Complex-Associated Angiomyolipomas (TSC-AML). Urology 2016;89:19-26.
- Mullerad M, Hidas G, Kastin A, Issaq E, Moskovitz B, Nativ O. Nephron sparing surgery as a treatment modality for re- nal angiomyolipoma. Harefuah 2005;144:619-21.
- Krueger DA, Northrup H. Tuberous Sclerosis Complex Surveillance and Management: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013;49:255-65.
- Yılmaz E, Özdemir K, Başaran C, Gözmen ŞK, Erturgut P, Serdaroğlu E. Kidney involvement in tuberous sclerosis complex. Dicle Med J 2015;42:66-71.
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Bölüm | Case Report |
| Yazarlar | |
| Yayımlanma Tarihi | 1 Aralık 2016 |
| Yayımlandığı Sayı | Yıl 2016 Cilt: 26 Sayı: 4 |
Genel Tıp Dergisi Creative Commons Atıf-GayriTicari 4.0 Uluslararası Lisansı (CC BY NC) ile lisanslanmıştır.