Giant bleeding renal angiomyolipoma: Diagnosis and management

Ashebu, S. D.; Dahniya, M H; Elshebiny, Yahaya H; Varró, J; Al-Khawari, Hana

doi:10.1046/j.1440-1673.2001.00969.x

Cited by 5 publications

(7 citation statements)

References 32 publications

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“…The second, isolated type (80%) are single asymptomatic lesions with a female/male ratio of 4:1 and usually occur in women 50-60 years of age. Renal AMLs may be detected in 40-80% of patients with tuberous sclerosis (1).…”

Section: Discussionmentioning

confidence: 99%

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Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report

Baran¹,

Aykaç²,

Öner³

et al. 2018

uob

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Angiomyolipoma (AML) is a mesenchymal tumor of the kidney that is composed of morphologically abnormal smooth muscle cells, blood vessels, and adipose-like foci. Renal AML is usually clinically asymptomatic and detected incidentally during imaging. Rarely, renal AML can cause life-threatening spontaneous massive retroperitoneal hemorrhage, known as Wunderlich syndrome. A 39-year-old man with tuberous sclerosis was admitted to the emergency room with left flank pain, hematuria, and nausea. On physical examination, there was a hard sensitive mass extending from the upper left half of the abdomen to the midline. Left renal AML and extensive retroperitoneal hematoma measuring about 360x220x195 mm were detected on abdominal computed tomography. The patient exhibited signs of hypovolemic shock and emergency total nephrectomy was performed. He was discharged from the intensive care unit on postoperative day 1 and from the hospital on day 5.

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Section: Discussionmentioning

confidence: 99%

“…Renal angiomyolipoma (AML) is a mesenchymal tumor of the kidney that is composed of morphologically abnormal blood vessels, smooth muscle cells, and adipose-like foci (1). Renal AML is usually clinically asymptomatic and may be detected incidentally during imaging.…”

Section: Introductionmentioning

confidence: 99%

Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report

Baran¹,

Aykaç²,

Öner³

et al. 2018

uob

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“…Ancak tipik bulgular, yağ içermeyen AML, hemoraji veya nekroz durumunda değişebilir. Böyle durumlarda kitleyi diğer solid tümörler-den ayırt etmek mümkün olmayabilir (1). Bizim olgumuzda, karın US'de geniş boyutlu ekojen kitle lezyonları, sağda kitle içinde ve çevresinde kan lehine değerlendirilen yoğun içerikli sıvı değerleri izlendi.…”

Section: Discussionunclassified

“…Bilateral olan olgularda nefrektomiden kaçınılsa da, özel-likle hemoraji kontrolü amacıyla nefrektomi gerekebilir. Alternatif yaklaşımlar elektif arteriyel embolizasyon ve parsiyel nefrektomidir (1,2). Sonuç olarak; tüberosklerozda nadiren geniş boyutlarda renal AML'ler görülür.…”

Section: Discussionunclassified

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Tüberosklerozda dev renal anjiyomyolipomlar: Bilgisayarlı tomografi bulguları Giant Renal Angiomyolipomas in Tuberous Sclerosis: Computed Tomography Findings

Nisa¹

2005

Ankara Üniversitesi Tıp Fakültesi Mecmuası

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Tüberoskleroz, tüm organları tutabilen ancak en sık deri, beyin, kalp, göz ve böbreği tutan benign hamartomatöz lezyonlar ile karakterize otozomal dominant bir hastalıktır. Tüberosklerozluların %40-80'inde bilateral, multipl, küçük ve sıklıkla asemptomatik renal anjiyomyolipomlar görül-mektedir. Ancak sunulan olguda olduğu gibi boyutları 20 cm'yi aşan anjiyomyolipomlar oldukça nadirdir. Boyutları büyüdükçe semptomatik olma olasılığı ve masif, hatta fatal hemorajiyle sonuçlanan spontan rüptür riski artmaktadır. Bilgisayarlı tomografi, düşük oranlardaki yağ içeriğini bile saptayabilmesi nedeniyle, renal anjiyomyolipomların tanısında ilk tercih edilen görüntüleme yöntemidir. Anahtar sözcükler: anjiyomyolipom, bilgisayarlı tomografi, tuberosklerozTuberous sclerosis is an autosomal dominant disorder characterized by hamartomas that may occur in any organ system, but most often occur in skin, brain, heart, eye and kidney. Between 40 and 80% of patients with tuberous sclerosis have renal angiomyolipomas which are often bilateral, multifocal, small and often asymptomatic. Very large renal angiomyolipoma, like the case reported here, are very rare. They are often symptomatic and have a propensity for spontaneous rupture, resulting in massive and at times, fatal hemorrhage. Because of its sensitivity in detecting small amounts of fat, computed tomography is imaging modality of choice in the diagnosis of renal angiomyolipomas.

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Can we predict the existence of extrarenal feeders to renal angiomyolipomas?

et al. 2018

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To identify factors predicting the presence of extrarenal feeders to renal angiomyolipomas (AMLs). MethodsThis is a retrospective study of 44 patients with 58 renal AMLs embolized in our department. Arteriography obtained during embolization and CTA obtained before and after embolization were reviewed to characterize AMLs with and without extrarenal feeders. Tumor characteristics were compared between the two groups.Simple logistic regression and ROC curve analysis were performed. P < 0.05 was considered to be statistically significant. ResultsOf the 58 AMLs reviewed, 29% had extrarenal arteries and 71% did not. AMLs with extrarenal feeders were significantly larger than those without, in terms of volume (median, 368 mL versus 109 mL, p < 0.0002) and largest diameter (mean, 12.0 cm versus 7.7 cm, p < 0.0001). Patient age, presence of tuberous sclerosis complex or sporadic lymphangioleiomyomatosis, and tumor location did not differ between the groups. Largest diameter and volume had similar predictive values for the presence of extrarenal feeders (AUC, 0.83 versus 0.82, p = 0.673). Extrarenal feeders were present in 0%, 21%, and 79% of the AMLs ≤6.5 cm, AMLs 6.6-10.5 cm, and AMLs >10.5 cm, respectively. ConclusionsAML size correlates with the presence of extrarenal feeders, with largest diameter and volume being significant predictors. AMLs >10.5 cm had a high chance of extrarenal feeders, making it mandatory to search for feeders to them in order to avoid incomplete embolization; AMLs ≤6.5 cm did not have extrarenal feeders, making a search for them unnecessary in these cases.

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Giant bleeding renal angiomyolipoma: Diagnosis and management

Cited by 5 publications

References 32 publications

Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report

Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report

Tüberosklerozda dev renal anjiyomyolipomlar: Bilgisayarlı tomografi bulguları Giant Renal Angiomyolipomas in Tuberous Sclerosis: Computed Tomography Findings

Can we predict the existence of extrarenal feeders to renal angiomyolipomas?

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