Nazir, Zafar

Talati, Jamsheer J

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eCommons@AKUSection of Urology Department of SurgeryJuly 1992Renal aRENAL ANGIOMYOLIPOMA: ANUNCOMMON TUMOURZafar NazirAga Khan University, zafar.nazir@aku.eduJamsheer J TalatiAga Khan University, jamsheer.talati@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_urolPart of the Urology CommonsRecommended CitationNazir, Z., Talati, J. (1992). Renal aRENAL ANGIOMYOLIPOMA: AN UNCOMMON TUMOUR. Journal of Pakistan MedicalAssociation, 42(7), 163-165.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_urol/72RENAL ANGIOMYOLIPOMA: AN UNCOMMON TUMOURPages with reference to book, From 163 To 165 Zafar Nazir, Jamsheer Talati ( Department of Surgery, The Aga Khan University Hospital, Karachi. ) The term angiomyolipoma of the kidney was first introduced by Morgan et al1 to denote an uncommontype of renal tumour with the histological components of thick-walled vessels, smooth muscle cellsextending outwards from the vessels in a peritheliomatous fashion and fat cells of the adult and foetaltypes. Although the tumour is referred to as a renal hamartoma2, a mass containing tissues normallypresent in the organ but abnormal in their arrangement, in fact, renal angiomyolipoma (HAML) arechoriostomas, as the fat is not the normal component of the human kidney3. Isolated angiomyolipomasare relatively rase renal tumours. Till 1988 only 250 cases were reported in English literature. Thepresent study adds 3 additional cases and review the outstanding clinical features and advances in thediagnosis and management. Case 1. L.M.N. a 47 year old diabetic, hypertensive female presented in theoutpatient clinic for evaluation for right sided flank pain of 5 months duration. There was no history ofbowel or urinary symptoms. Examination revealed a tender, firm and smooth mass measuringapproximately 20x15 cm occupying the right side of the abdomen. CBC, urine analysis, 24 hoururinary VMA and biochemical profile were normal. The abdominal ultrasonography and intravenousurography revealed a space occupying lesion in right kidney and fullness of the pelvicalyceal system.The CAT scan revealed a 9 cm nonhomogeneous mass with areas of low attenuation values (-65hounsfields units) in the right kidney (Figure 1).In addition a 2.5 cm solid mass arising from the right adrenal gland was noted. There was no evidenceof retroperitoneal lymphadenopathy. Renal cell carcinoma could not be excluded on CT scanappearances. At exploratory laparotomy after opening the gerota’s fascia a tumour mass was seenwhich was mostly extrarenal but was growing into the right kidney, leaving only a thin rim of cortex. Itwas soft and spongy to feel and was not vascular. A separate adrenal mass hard in consistency couldalso be felt. The tumour mass, right kidney and adrenal gland were excised in toto. Histopathologyconfirmed angiornyolipoma of kidney and adrenal gland. The postoperative course was uneventful andthe patient has remained well and symptom free throughout a two years follow- up. Case 2. S.F.N. a 45year, hypertensive female presented with left lumber pain of one year duration. Her symptoms hadworsened since the past month and had become associated with generalized weakness, anorexia, nauseaand vomiting. There was no history of fever and urinary symptoms. Examination revealed pallor and abimanually palpable mass measuring approximately 30x20 cm in left lumber region. Hemoglobin was9.2 grn/dl with hcmatocrit 27.7%. A urinalysis and routine biochemical profile was normal. Anintravenous pyelogram revealed an enlarged left kidney with adequate excretion of dye. An ultrasound(US) of the abdomen showed ahyperechoic mass arising from lateral aspect of left kidney. CT scan ofabdomen showed a perinephric hematoma and an intrarenal mass measuring 6x6cm, showingdecreased attenuation values (-60 hounsfield units) supportive of fat in the kidney. A renal angiographydemonstrated a nonvascular mass, multisaculated aneurysmal dilatation of peripheral artery, sunburstappearance of veins and lack of arteriovenous shunting (Figure 2).A preoperative diagnosis of RAML was made. Exploratory laparotomy revealed a large perirenalhematoma contained by the gerota’s fascia. Evacuation of hematoma revealed a 6x6 cm, welldemarcated, yellowish firm mass arising from the lateral aspect of upper pole of left kidney.Hemostasis was secured by multiple suture ligation and the cavity was obliterated with an omentalpedicle. The diagnosis of RAML was confirmed on frozen section and again on final histopathologicalexamination (Figure 3).Postoperative recovery was uneventful. The patient has remained symptom free throughout the 18months follow-up. Case 3. K.A. 34 year old female who was previously in good health presented to theemergency room with acute flank pain associated with nausea, vomiting and sweating. There were nourinary or bowel symptoms. Examination revealed a tender, firm mass measuring approximately 15x10cm occupying the right side of abdomen. Hemoglobin was 5.1 gm/dl with hematocrit 16.8%. Urinalysisrevealed microscopic hematuria. Biochemical profile was normal. Intravenous pyelogram andultrasound of abdomen revealed a space occupying lesion in the right kidney. CT scan showed perircnalhaematoma, a 4 cm smooth mass in the right kidney and a huge retroperitoneal mass with lowattenuation value (-65 negative hounsfield units) consistent with diagnosis of RAM!.. The patientnormally a resident of London, returned there where she underwent a right nephrectomy.Histopathology revealed RAML.DISCUSSION BAML are uncommon benign lesions that may occur as an isolated phenomenon or as seen in 20-50%of patients as part of the syndrome associated with tuberous sclerosis (Bournvill’s disease4). About 50-80% of patients with tuberous sclerosis will have angiomyolipoma5. Therefore these patients requireperiodic screening as these can be occasionally symptomatic6. RAML as an isolated phenomenonusually present as a large symptomatic unilateral mass. They occur predominantly in women in fifthand seventh decades7. Grossly RAML are greyish-yellow in colour and have propensity for profusehemorrhage and local aggressive growth7. They may occasionally parasitize to external blood vesselsand lymph nodes. The actual incidence of nodal involvement is not known. A review of literaturereveals only 8 cases9. The involvement of regional lymph nodes and blood vessels has been attributedto the multicentric origin of the lesion rather than metastatic bchaviour10-13. Todate there has been nodeath due to mctastatic spread. Microscopically angiomyolipoma consists of sheaths of smoothmuscles, abnormal thick wall blood vessels and clusters of adipocytes in varying proportions andarrangements (Figure 3). Renal cell carcinoma may coexist with angiomyolipoma in the same kidney.Twelve such cases until 1989 have been reported in literature9,14,15. One case of simultaneous RAMLand oncocytoma has also been reported16. The clinical presentation varies. Symptoms may be absent ifthe lesion is small (<4cm). When present. symptoms are usually a result of intrarenal or perirenalhemorrhage. More than 50% of patients with angiomyolipomas larger than 4cm have associated hemor-rhage12. The most frequent signs and symptoms are acute flank/abdominal pain, palpable mass,hcmaturia, shock and anaemia. Hypertension and fever has been reported8,12. The diagnosis ofangiomyolipoma should be borne in mind since 25% of patients with RAML described in the literaturepresented with severe abdominal pain with or without shock, which necessitated emergencylaparotomy8. In the past, correct preoperative diagnosis has been extremely difficult with the result thatmost patients undergo nephrectomy for a mistaken diagnosis of renal cell carcinoma1,3,12. In recentyears CT scan and ultrasonography have resolved the diagnostic dilemma, making definitive diagnosisof RAML possible in almost all cases8,13. Angiomyolipomas are the most echogenic form of renaltumours, due to their high fat content4. However this pattern is not always pathogonomonic since otherechogenic lesions, including renal cell carcinoma incorporating perirenal fat, liposarcoma, lipoma,teratoma, oncocytoma, cavernous hemangioma and renal sinus lipomatosis may also exhibit a similarpicture. A CT diagnosis of angiomyolipoma requires identification of fat component in the tumourwhich usually stands out in the lesion as areas with low attenuation value (negative hounsfield unit).Lesions smaller than 2.0 cm may be difficult to diagnose owing to partial volume effect. Moreover ahemorrhage within or outside the tumour may increase the attenuation value to above that of fat17. A50-78% diagnostic accuracy of CT scan has been reported1,3,8,12,13,17. We were able to make tentativepreoperative diagnosis in all of our cases with CT scan but were reluctant to confidently excludecarcinoma except in the case who had angiography (case No.2). Sincc CT scan cannot consistentlydiagnose all EAML, a fine needle aspiration biopsy (FNA) has been advocated by Scant andassociates18 to solve the diagnostic dilemma18. Selective renal angiográphy has been suggested bothfor diagnostic and therapeutic purposes12. The angiographic characteristic of an angiomyolipoma arehypovascular mass with circumferential tortuous dilated peripheral vessels having multiple aneurysms,sunburst or whorled appearance of the veins and lack of the arteriovenous shunting which is seen inrenal cell carcinomas19-21. Selective arterial embolization has been shown to be effective in treatingacute hemorrhage alone or in conjunction with surgical intervention4. With accurate preoperativediagnosis of the angiomyolipoma, most urologists currently recommend a conservative approach to themanagement. The nephrectomy rate has declined from 93% in 1976 to 50% in 198412. For small (<4cm) asymptomatic tumours, surveillance is all that is required. With large (>4cm) and symptomaticangiomyolipomas, renal conservation with tumour excision or partial nephrectomy are preferred ifpossible1,3,4,7,8,12. However it is important that conservation should never compromise completeness.Frozen section may be helpful for the diagnosis of coexistent renal cell carcinoma12. In conclusion,renal angiomyolipoma, although a rare lesion, should be included in the differential diagnosis, whendealing with a patient presenting with severe flank pain and anaemia. Considering the benign nature ofdisease and improvement in preoperative diagnosis, a kidney conserving operation is desirable.ACKNOWLEDGEMENTS We wish to thank Dr. Hizbullah Sheikh, Pathology Department, The Aga Khan University Hospital forproviding the pathological material and Mr. Muradali Bana for secretarial help.REFERENCES 1. Tong. Y.C., Chieng. P.U., Tsai. T.C. el at. Renal angiomyolipoma. Report of 24 cases. Br.J.Urol.,1990;66:586-89.2. Case records of the Massachusetts General Hospital (Case No.51. 1985). N.Engl.J.Med..1985;313:1596-1603.3. Ahmed, I.. Multi, A.B.. Sebayel, M.l. et al. Renal angiomyolipoma. Report of two cases andreviewof literature. Em.Med.J., 1989;7:100-104.4. Johnson. D.E.,Swanson, D.A. and Eschenbach, A.C.V.Tumoursofthegenitourinary tract, in Smith’sgeneral urology. Edited by K.A. Tanagho and j.W. McAnirch. connecticut, Prentice-Hall, 1988, p.334.5. Stillwell, T.J.. Gomez, M.R. and Kelalis, PP. Renal lesions in tuberous sclerosis. J.Urol.,1987;138:477-81.6. Hendren, W.G. and Monfort, Gi. Symptomatic bilateral renal angiomyolipoma in a child. J.Urol.,1987;137:256-57.7. Dekernion, J.B. Renal tumours, in Campbell\'s urology. Edited by P.C. Walsh et al Philadelphia.Saunders, 1986, vol.2, pp. 1294-1342.8. Blute, ML, Mulek, R.S. and Segura, 3W. Angiomyolipoma: Clinical metamorphosis and concepts formanagement. J.Urol.,1988; 139:20-24.9. Taylor, R.S., Joseph, D.B., Kohat, E.C., Wilson, ER. and Bueschen, AJ. Renal angiomyolipomaassociated with lymph node involvement and renal cell carcinoma in patients with tuberous sclerosis.J.Urol., 1984;14 1:930-32.10. McIntosh, G.S., Dutoit, H., Chronos. N.y. and Kaisary, A.V. Multiple unilateral angiomyolipomaswith regional lymphangiomyomatosis. J.Urol.,1989;142: 1305.7.11. Castillenti, J.A. and Bertin, A.?. Angiomyolipoma of the spermatic cord. Case report and literaturereview. J.Urol., 1989;142:1308-9.12. Oesterling. J.E.. Fishman. K.E., Goldman, SM. and Marshall. F.F. The management of renalangiomyolipoma. J.Urol., 1986; 135:1121-4.13. Rothenberg. D.M., Brandt, T.D. and- D\'Cruz, I. Computerized tomography of renalangiomyolipoma presenting as right strial mass. J. Compact.Assist.Tomogr.,1986;10: 1054.56.14. Huang. J., Ho D.M., Wang. .1., Chou, Y., Chen, M. and Chang, S. Coincidental angiomyolipomaand renal cell carcinoma ‘Report of one case and review of literature. J.Urol.. l98$;140 l516-1815. Graves, N. and Barnes, W.F. Renal cell carcinoma and angiomyolipoma in tuberous sclerosis: Casereport. J.Urol., 1986;135: 122-23.16. Waters, DJ.. Holt, S.A. and Andres, D.F. Unilateral simultaneous renal angiomyolipoma andoncocytoma. J.Urol., 1986;135:568-7017. Bosniak. M.A., Megibow. A.J., Hulnick, D.H.. Horii. S. and Raghavendra, B.N. CT diagnosis ofrenal angiomyolipoma: the importance of detecting small amount of fat. AJR., 1988;151:497-501.18. Sant, G.R.. Ayers. D.K., Bankoff, M.S., Mitcheson. H.D. and Crei, A.A.Jr. Fine needle aspirationbiopsyin thediagnosisof renal angiomyolipoma. J.Urol., 1990;143:999-1001.19. Peary, N.M., Webb. J.A.W., White, FE. - and Whitfield. H.N. Hemorrhagic angiomyolipoma of thekidney. J.Urol., 1984;132:749. 51.20. Davis, T.J. Parasitic arterial supply to renal angiomyólipoma. J.Urol., 1978;118:271-7421. Barzilai, T.M., Braden, G.L., Ford, L.D., Goodman, LH., Delima, R.J.. Germain, M.J. andFitzglhbons. J.P. Renal angiomyolipoma with arteriovenous shunting. J.Urol., 1987;137:4838-4.

Renal angiomyolipoma: An uncommon tumour

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Renal angiomyolipoma: An uncommon tumour

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