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Spontan Anjiomyolipom Rüptürüne Bağlı Akut Retroperitoneal Hematom:Olgu SunumuİSpontaneus Rupture of Renal Angiomyolipoma As a Cause of AcuteRetroperitoneal Hemorrhage: Case ReportOlgu Sunumu

Başvuru: 27.12.2014Kabul: 01.02.2015Yayın: 20.03.2015

Fulya Yılmaz Duran1, Özgür Duran2, Umut Öztürk2, Gizem Demir1, Zafer Kozacıoğlu3, İsmail Gülden 3,Yasin Ceylan3

1 Bozyaka Training and Research Hospital, Anaesthesiology and Reanimation Department2 Bozyaka Training and Research Hospital, Emergency Medicine Department

3 Bozyaka Training and Research Hospital, Urology Department

Özet

Anjiomyolipom, yağ dokusu, düz kas hücre demetleri vekalın duvarlı kan damarlarının karışımından oluşan,nadir rastlanan mezenkimnal bir tümördür. 35 yaşındakibayan hasta nedeniyle sol yan ağrısı, bulantı ve kusmaacil servisine başvurdu. Fizik muayenede yüzündeadenom sebaseum gibi deri lezyonları saptandı.Bilgisayarlı tomografide sağ alt kutupta yağ densitesindealanlar (35mm) ve ekzofitik kitle lezyonu saptandı. acillaparatomi uygulandı. Renal anjiomiyolipomdrüptürünebağlı retroperitoneal kanama gözlendi. Bir gün sonrayoğun bakım ünitesinden ve bir hafta sonra hastanedentaburcu edildi. Patoloji raporu anjiomyolipom rüptürü ileuyumlu saptandı.

Abstract

Angiomyolipoma is a rare mesenchymal tumorcomposed of the mixture of mature fat tissue, smoothmuscle cell bundles and thick-walled blood vessels. A35-year-old woman was admitted to emergencyservice because of left flank pain, nause and vomiting.Physical examination revealed skin lesions on her facelike adenoma sebaceum. Computed tomography of theabdomen revelaed areas with fat density at the lowerpole of the right kidney (35mm) and exophytic renalmass lesions. She underwent emergency laparatomy.Retroperitoneal hemorrhage due to renalangiomyolipoma rupture was observed. The patientdischarged from ICU unit one day later and fromhospital one week later. Pathology report declaredangiomyolipoma.

Anahtar kelimeler: Anjiomyolipom, Spontan rüptür,Retroperitoneal hemoraji

Keywords: Angiomyolipoma, Spontaneous rupture,Retroperitoneal hemorrhage

Introduction

Angiomyolipoma is a rare mesenchymal tumor composed of the mixture of mature fat tissue, smooth muscle cellbundles and thick-walled blood vessels. Although often benign, some types are considered as malign. [1-6]. Twotypes were described: sporadic or associated with tuberosclerosis [4,5]. 40% of patients are symptomatic [5].Clinically symptoms are palpable abdominal mass, hematuria, flank pain [1,3,5-7]. We report a case who underwentemergency laparotomy due to massive retroperitoneal hemorrhage because of anjiomyolipoma rupture.

Case Report

A 35-year-old woman was admitted to emergency service because of left flank pain, nause and vomiting. She wastransferred to our emergency department from emergency room of other hospital for acute left flank paininterpreted as a renal colic and not responsive to medical therapy. She has no feature in her and family history. Onclinical examination she was hemodynamically instable with rapid decrease in hemoglobin (Hb: 6.7mg/dL), with

Sorumlu Yazar: Fulya Yılmaz Duran, Bozyaka Training and Research Hospital, Anaesthesiology and Reanimation DepartmentSaim Çıkrıkçı Caddesi No. 51drfulya@mynet.com

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a persistant left flank pain and hypochondrium pain as in acute abdomen. Blood pressure:80/40 mmHg, heartrate:135/min. Physical examination revealed skin lesions on her face like adenoma sebaceum. Abdominalultrasonografi showed hyperechoic lesions around the left kidney (the largest is 12cm diameter), suspectedhematoma around the left kidney, hyperechoic lesions with the same characteristics like on the left kidney on thelower pole of the right kidney (the largest is 2cm diameter), abdominal free fluid. Subsequent computedtomography of the abdomen revelaed areas with fat density at the lower pole of the right kidney (35mm) andexophytic renal mass lesions. The appearance which suggested bleeding was detected in the left kidney, whichwas more pronounced in the lateral segment and enlargement of the left psoas muscle was revealed. Free fluidviewed in the pelvic area and abdomen. The patient was consulted by general surgery and urology doctors. Sheunderwent emergency laparatomy. Retroperitoneal hemorrhage due to renal angiomyolipoma rupture wasobserved. Left radical nephrectomy was done. The patient was stabile in perioperative course. 4U of packed redcells transfused during the operation. She was transported to intensive care unit(ICU) after the operation. Thepostoperative period was uneventful. The patient discharged from ICU unit one day later and from hospital oneweek later. Pathology report declared angiomyolipoma. Tuberousclerosis was not detected in the genetic analysis.This case report was reported after the patient approvel was taken when she was discharging from the hospital.

Discussion

Spontaneous angiomyolipoma rupture was occured in our case. The patient was underwent emergencynephrectomy with early diagnosis. Lesions at right kidney were followed up with computure tomography.

The term angiomyolipoma was first used by Morgan et al at 1951 to define the kidney tumors composed ofaberrant vascularization with varying amounts of smooth muscle and fatty elements [5] 1-2% in the generalpopulation [7] , often frequently seen in middle-aged women [6,7]; 50-75% seen in patients with tuberous sclerosisand at a younger age, without distinction of sex arises [7].

Anjiomyolipoms that <4 cm are often asymptomatic [1,2,6]. Sometimes classical Lenks triad occurs:massproportional to the diameter of lesion, flank pain and hematuria [1,3,5-7]. Massive retroperitoneal haemorrhage dueto AML (Wunderlich syndrome) was 10% in cases and the most feared complication [1,7].

In our case, at the first examination lumbar pain and non-specific symptoms such as nausea and vomiting werepresent. hypotension and tachycardia were present. Hemogram revealed a decrease in hemoglobin levels duringfollow-up. As a result of the tests performed emergency laparotomy was decided.

In most cases, angiomyolipoma is diagnosed with typical radiological findings [1]. CT is the most reliablediagnostic tool [1,3]. In our case, USG detected suspicious lesions and the possibility of bleeding and the diagnosiswas confirmed by CT.

At angiomyolipoma, the walls of arteries are thicker than normal renal vessels but has less elasticity [1]. They arewilling to bleeding without trauma or minor trauma. The risk of bleeding is increasing with 4 cm or greaterdiameter [1,5-8]. Spontaneous rupture is a serious medical condition.[1] In our case, there is no history of trauma.Spontaneous rupture was considered. Sudden onset of flank pain, nausea, and hypotension was revealed.

The treatment in asymptomatic angiomyolipoma is conservative treatment and intermittant follow upexamination [1,7,8]. In patients with symptoms, the aim is to protect the nephrons and renal function [1,7]. Firstly,selective arterial embolization, open or laparoscopic partial nephrectomy, open or laparoscopic cryoablation orradiofrequency ablation, and total nephrectomy should be performed if necessary [2,3,7].

In our case, the result of spontaneous rupture disrupts hemodynamic parameters. We did not have time to

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implement selective uterine artery embolization. Primarily to protect the nephrons partial nephrectomy wasplanned but has been forced to total nephrectomy. Active pathology in the right kidney was not observed.

In the literature, spontaneous rupture of renal anjiomyolipom at 31 year old woman during pregnancy have beenreported. It is a dangerous condition that may caause mortality in the mothr and fetus. Elective simultaneouscesarean section and radical nephrectomy cen be performed safetly if hemodynamic status is stable [9].

Consequently, AML often accompany tuberousclerosis, but also available spontaneously. The most importantcomplication is massive retroperitoneal hemorrhage. While choosing treatment methods, renal functions shouldbe preserved maximally. In the emergency room with a diagnosis of renal colic patients are advised to check withthe ultrasonography and computure tomography.

References

1. Gül AB, Sudutan V, Altunoluk B, Şekkeli K. The rupture of renal angiomyolipoma caused by minortrauma. Kartal Eğitim ve Araştırma Hastanesi Tıp Dergisi 2009;(2):95-98.

2. Lucky MA, Shingler SN, Stephenson RN. A case report of spontaneous rupture of a renalangiomyolipoma in a post-partum 21-year-old patient. Arch Gynecol Obstet 2009;280(4):643-5.

3. Semerciöz A, Atikeler B, Yekeler H, Serhatoğlu S, Baydinç C. A cause of hemorrhagic shock: Bilateralangiomyolipoma with tuberous sclerosis (A case report). Türk Üroloji Dergisi 1996;22(4):493-496.

4. Yıldız E, Tuncer E, Gökçe G, Kılıçarslan H. Renal angiomyolipoma with predominant spindle cellcomponenet: Report of two cases. Turkısh Bulletin of Pathology 2002;19 (2):85-89.

5. Başpınar Ş, Bircan S, Bozkurt KK, Yavuz G, Tuncer SS, Oksay T, Koşar A. Renal angiomyolipoma:Report of two cases. Süleyman Demirel Üniversitesi Yaşam Dergisi 2010; 2(2):04-08.

6. Wang HB, Yeh CL, Hsu KF. Inter Med 2009:1111-1112.7. Kılıç Ö, Yurdakul T, Kaynar M, Özbek O, Baba F. Tubero Sklerozlu hastada bilateral renal

anjiomyolipom sebebi ile masif retroperitoneal kanama. Gazi Medical Journal 2009;20 (2):90-93.8. Furuset AS, Bjerklund Johansen TE, Majak B. Renal angiomyolipoma as a cause of acute retroperitoneal

hemorrhage. Tidsskr Nor Laegeforen 1997;117(29):4215-7.9. Koh JL, Lee YH, Kang CY, Lin CN. Simultaneous cesarean section and radical nephrectomy for

angiomyolipoma with spontaneous bleeding during pregnancy: a case report. J Reprod Med2007;52(4):338-40.

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