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Unformatted text preview: AnnalsoftheRoyalCollegeofSurgeonsofEngland(1992)vol.74, 144-148 Angiomyolipoma of the kidney: a clinical enigma in diagnosis and management Kit Wong MB BS FRCS SurgicalRegistrar Amir V KaisaryMS FRCS ConsultantUrologist UniversityDepartment ofSurgery, The Royal Free Hospital, London Catherine M Waters MB BS SurgicalHouse Officer Joseph Horner FCS(SA) FRCSEd ConsultantSurgeon Department ofSurgery, Ashford Hospital,Ashford, Middlesex Michael J Hershman MS FRCS SeniorRegistrarinSurgery StMary's Hospital, London Key words: Angiomyolipoma; Radicalnephrectomy; Renalpreserving surgery Renal angiomyolipomas are uncommon benign tumours which clinically mimic renal cell carcinoma. They have characteristicfeatures on ultrasoundandCT scanningwhich may enable their diagnosis preoperatively. We review our experience of six cases of renal angiomyolipoma. These cases highlight the difficulties in making a preoperative diagnosisdespitetheuse ofmodern scanning.We advocate conservativeresectionwhenthediagnosisismade preopera- tively and when technically possible, but radical nephrec- tomy should be performed if there is a possibility of a carcinoma. Renal angiomyolipomas are rare benign tumours com- posedofthick-walledblood vessels,fully differentiated smooth muscleand adiposetissue.Two distinctclinico- pathologicalentitiesexistdepending on theirassociation with tuberous sclerosis. When renal angiomyolipomas occur inpatientswith tuberous sclerosistheyare often bilateral,small,multipleand asymptomatic.Conversely, angiomyolipomasnot associatedwith tuberous sclerosis present as unilateral, large and symptomatic tumours. Preoperativediagnosisisoftendifficult as theycan mimic renal cellcarcinoma clinicallyand radiologically. More recently,refinementin computedtomographyandultra- sonographyhaveenabledaccurate preoperativediagnosis (1,2)and hasmade renal-preservingoperationspossible (3).We report sixpatientswith angiomyolipoma. None had stigmata nor family history of tuberous sclerosis. These cases illustrate the difficultyinmaking accurate preoperativediagnosisdespitetheuse ofmodern scan- nmg facilities. Case reports Case I A 63-year-old woman was incidentallyfound to have a rightloin mass by her generalpractitioner. She had no historyofloinpainor haematuria.Physicalexamination revealed a firm, mobile, non-tender 15 cm mass inthe right loin. An intravenous urogram confirmed a mass lesioninthelowerhalfoftherightkidney.An abdominal ultrasound revealed a hyperechogenic dumbell-shaped mass inthe kidney. Laparotomy revealed a large mass arisingfrom thelowerpoleoftherightkidneywith no evidenceofmetastases. A rightradicalnephrectomywas performed. Macroscopic examination revealed three nodular tumours in the lower pole ofthe rightkidney measuring 3 cm x 2 cm, 4 cm x 3 cm and 12 cm x 9 cm x 8cm. Histological appearances were consistent with angiomyolipoma. The patientremainswell5years later....
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- Spring '09
- Renal cell carcinoma, Medical ultrasonography, angiomyolipomas