FROM THE KLINIK FUR RADIOLOGIE UND NUKLEARMEDIZIN 1M KLINIKUM STEGLITZ DER FREIEN UNIVERSITAT BERLIN, D-IOOO BERLIN, GERMANY.

ADVANCES IN THE DIAGNOSIS OF RENAL ANGIOMYOLIPOMA D. E. APITZSCH, a.-H. WEGENER, M. KHALIL and R. SORENSEN Since the angiographic appearance of angiomyolipoma was first described by Dos SANTOS & WOHLWILL (1942) increased experience has shown that difficulties in distinguishing this benign tumour from a renal carcinoma are often encountered. While some authors (VIAMONTE et colI. 1966, KHILNANI et colI. 1968, BARRILERO et coIl. 1974, BARRILERO 1977) are of the opinion that the benign tumour has special angiographic characteristics, others such as KAUDE & CHANG (1970) emphasize the impossibility of excluding a renal carcinoma preoperatively. For practical clinical purposes the angiography may occasionally allow a tentative diagnosis of a hamartoma. However, the vast majority of these tumours will be determined only after microscopic examination. The rare angiomyolipoma consists of individually differing zones of fat, smooth muscle and angiomatous tissue. In 50 to 80 per cent of cases it is detected as a lesion combined with tuberous sclerosis (Bourneville-Pringle disease) belonging to the group of phacomatoses. Frequently, the tumour is bilateral; total involvement of the kidneys is rare (KLAPPROTH et coIl. 1959, ANDERSON & TANNEN 1969, HEMMATI & THIELE 1970, APITZSCH & KALK 1974). Recognition of these tumours without concomitant tuberous sclerosis only occurs by chance (Case 2). Up to the present time the radiologic diagnosis has been based upon conventional films of the kidney, at urography and at nephroangiography. Submitted for publication 26 April 1978. Acta Radiologica Diagnosis 20 (1979) Fasc. I A

Downloaded from acr.sagepub.com by guest on July 25, 2015

105

106

D. E. APITZSCH, O.-H. WEGENER, M. KHALIL AND R. SORENSEN

Fig. 2

Fig. 1 Fig. 1. Intravenous urography. Right renal pelvis (-) displaced towards the left side by a large expanding lesion with attenuation properties similar to fat. Fused vertebrae of the lumbar spine. Fig. 2. Same case as in Fig. 1. Selective angiography. Double renal collecting system with tumour in the larger part of the right kidney. Multiple aneurysms, some arranged in clusters. The tumour region is still in the arterial phase while the rest of the kidney is in the parenchymal phase.

Fig. 3. Selective angiography. Hypovascular tumour with neovascularity. No cortical spur formation. Fig. 3

An expanding lesion in the kidney, in some regions with an attenuation similar to that of fat, is characteristic (PALMISANO 1967, BARON et coli. 1977). Such an appearance may, however, also be observed in a lipoma (WINDHOLZ 1946). BARON et colI. detected lipid regions in 5 of 6 angiomyolipomas retrospectively, while 10 renal carcinomas did not have fatty regions microscopically. The angiographic characteristics of the angiomyolipoma as reported in the literature by different authors are as follows:

Downloaded from acr.sagepub.com by guest on July 25, 2015

RENAL ANGIOMYOLIPOMA

107

1. Hypervascular tumours with normal interlobar arteries (PALMISANO, HARTEL et coIl. 1973, OWMAN 1973). 2. Macro- and microaneurysms, occasionally arranged in clusters (LOVE & FRANK 1965, VIAMONTE et coll., KHILNANI et coll., SEABURY et colI. 1969, ISFORT & RINSCHE 1970, KAUDE & CHANG, BIGOT et colI. 1971, BRENDLER et colI. 1971, DEININGER & TRAPP 1971, JORG 1971, MCCULLOUGH et coil. 1971, CLARK & PALUBINSKAS 1972, BARRILERO et coil., BARRILERO). 3. 'Onion skin' in the venous phase (LOVE & FRANK, LEMAITRE et colI. 1968, MEANEY 1969, SEABURY et coil., BRENDLER et coil., DEININGER & TRAPP, MCCULLOUGH et coll., SILBIGER & PETERSON 1971, CLARK & PALUBINSKAS). 4. Multiple cyst-like regions in the parenchymal phase corresponding to the fatty part of the tumour (VIAMONTE et coli., PALMISANO, MEANEY, SEABURY et coll., BIGOT et coll., HARTEL et coll., OWMAN 1973). 5. Prolonged arterial phase and absence of arteriovenous shunts in spite of hypervascularisation (JORG, HARTEL et coll., BARRILERO et coll., OWMAN 1973, BARRILERO).

A differential diagnosis is impossible when the angiomyolipoma is hypovascular and without aneurysms, and if a renal carcinoma has a similar vascularity to a hamartoma (MEIISEL & APITZSCH 1978). In the only case of renal carcinoma and hamartoma of the same kidney, the malignant tumour could not be differentiated from the benign tumour even in retrospect (KAVANEY & FIELDING 1975). Computer tomography provides possibilities to facilitate a preoperative diagnosis by its greater capacity for recording attenuation differences. Angiography and computer tomography have been performed in 2 cases of angiomyolipoma at this hospital. As the appearances at computer tomography seem never to have been described before, a brief report seems motivated.

Case reports Case 1. A 47-year old female was admitted because of sudden severe pain in the upper abdomen. At the site of distress a mass was palpated. At urography, displacement of the right kidney pelvis towards the left side was demonstrated. In the kidney region, structure indicating renal tissue and fat appeared. The origin of the space-occupying lesion could not be clearly identified. At abdominal angiography and selective nephroangiography a large tumour in a duplicated kidney was demonstrated on the right side. Several aneurysms were found, some of them cluster-like. The nephrographic effect was very irregular. The arterial phase appeared to be prolonged and no early draining veins were observed (Fig. 2). The angiographic diagnosis was possible angiomyolipoma. CT: Large expanding lesion originating in the right kidney and with well demarcated fatty tissue. Smooth borders to the surrounding area without any invasive growth into the adjacent organs. No regional lymph node metastases. The angiographically suggested diagnosis of angiomyolipoma was confirmed by CT. The attenuation values indicated fatty tissue in the right renal bed in the same area as observed

Downloaded from acr.sagepub.com by guest on July 25, 2015

108

D. E. APITZSCH, O.-H. WEGENER, M. KHALIL AND R. SORENSEN

Fig. 4. Same case as in Figs I and 2. Computer tomography. Fat containing mass on the right side. A larger remainderof and renal parenchyma (~) small well demarcated remnants.

Fig. 5. Same case as in Fig. 3. Computer tomography. Rounded mass in the tilted right kidney with attenuation values similar to fat. Inconspicuous retroperitoneal structures. on the conventional abdominal film. The diagnosis of an angiomyolipoma was confirmed at microscopy. No abnormalities indicating tuberous sclerosis were found. Case 2. In a 65-year old female with chronic atheromatous vascular disease in both legs an abdominal aortography was performed. As an incidental finding there was a hypovascular space-occupying lesion in the lower pole of the right kidney (Fig. 3). The patient had no history of renal disease. The selective angiography demonstrated a hypovascular tumour with sparse neovascularity, which was considered to be a renal carcinoma (Fig. 5). No regions indicating fatty tissue. At CT a mass, 5 ern x 6 cm with smooth borders to the parenchyma, was detected. Septal structures crossed the perirenal space. The attenuation values indicated fatty tissue. No enlarged regional lymph nodes. Diagnosis: Fatty tumour of the right kidney without signs of malignancy, possibly angiomyolipoma, which was confirmed at microscopy. No indication of tuberous sclerosis.

Discussion. The 2 cases well elucidate the diagnostic advantage achieved by CT. In both cases, one examination, angiography or computer tomography, was not

Downloaded from acr.sagepub.com by guest on July 25, 2015

RENAL ANGIOMYOLIPOMA

109

sufficient to determine the true nature of the tumour. Only by comparing the results of the two methods could a definite diagnosis be made. However, further experience is necessary for a clear evaluation of the value and accuracy of the diagnosis of renal angiomyolipoma with combined CT and angiography.

SUMMARY Two cases of renal angiomyolipoma (benign hamartomas) are presented. These rare tumours were preoperatively diagnosed by comparing the conventional film, angiography and computer tomography.

ZUSAMMENFASSUNG Zwei Falle von renalen Angiomyolipomen (benignen Hamartomen) werden vorgesteIlt, in denen der Vergleich von Abdomenleeraufnahme, Angiographie and Computertomographie die praoperative Diagnose dieser seltenen Tumoren ermoglichte,

RESUME Les auteurs presentent deux cas d'angiornyolipomes renaux (hamartomes benins); la comparaison de la radiographie de l'abdomen sans preparation, de l'angiographie et de la tomodensitometrie a perm is le diagnostic pre-operatoire de ces tumeurs rares.

REFERENCES ANDERSON D. and TANNEN R. L.: Tuberous sclerosis and chronic renal failure. Potential confusion with polycystic kidney disease. Amer. J. Med. 47 (1969), 163. APITZSCH D. E. und KALK J.-F.: Beidseitige Angiomyolipomatose der Nieren. Fortschr. Rontgenstr. 121 (1974), 518. BARON M., LEITER E. and BRENDLER H.: Preoperative diagnosis of renal angiomyolipoma, J. Urol. 117 (1977), 701. BARRILERO A. E.: Renal angiomyolipoma: A study of 13 cases. J. Urol. 117 (1977),547. - BORRUEL J.-L. S. et MARTINEZ-PINEIRO J. A.: Diagnostic entre l'adenocarcinome renal et l'angiomyolipome. J. Urol. Nephrol. 80 (1974), 665. BIGOT R., GOLDLUST D., HELENON CH., MICHEL J. R. et BENOZIO M.: Angiomyolipomes isoles du rein. J. Radial. Electrol. 52 (1971), 789. BRENDLER H., MAGUIRE J. W. and MITTY H. A.: Angiographic characteristics of renal angiomyolipoma. Brit. J. Urol. 43 (1971), 674. CLARK R. E. and PALUBINSKAS A. J.: The angiographic spectrum of renal hamartoma. Amer. J. Roentgenol. 114 (1972), 715. CROSETT JR A. D.: Roentgenographic findings in the renal lesion of tuberous sclerosis. Amer. J. Roentgenol. 98 (1966), 739. DEININGER H. K. und TRAPP P.: Angiographische und nuklearmedizinische Befunde der Nieren beim Pringle-Syndrom (Adenoma sebaceum). Fortschr. Rontgenstr. 114 (1971), 782.

Downloaded from acr.sagepub.com by guest on July 25, 2015

110

D. E. APITZSCH,

o-rr. WEGENER, M. KHALIL AND R. SORENSEN

Dos SANTOS R. e WOHLWILL F.: Lipo-mio-angioma partiamente sarcomatoso bilateral dos rins (arteriografia) com doenca questica dos pulmos (novo tipo de 'facornatoses'), (In Portuguese.) Lisboa rned. 19 (1942), 131. HARTEL M., WEHLIN L. und LIEDBERG C. F.: Zur radiologischen Differentialdiagnose hypervascularisierter Nierentumoren. Fortschr. Rontgenstr, 118 (1973), 498. HEMMATI A. und THlELE K. G.: Die angiographischen Nierenveranderungen beim Bourneville-Pringle-Syndrom. Dtsch. med. Wschr. 95 (1970), 1885. ISFORT A. und RINSCHE K. G.: Das Hamartom der Niere im Angiogramm. Zugleich ein Beitrag zur postoperativen Organangiographie. Fortschr. Rontgenstr, 112 (1970), 231. JORG H.: Nierenhamartome bei tuberoser Sklerose. Fortschr. Rontgenstr, 114 (1971),381. KAUDE J. and CHANG T. T.: Angiography in evaluation of tuberous sclerosis complex. Radiologe 10 (1970), 105. KAVANEY P. B. and FIELDING J.: Angiomyolipoma and renal cell carcinoma in same kidney. Urology 5 (1975), 643. KHILNANI M., ABRAMS R. M. and BERANBAUM E. R.: Angiographic features of hamartoma of the kidney. Radiology 90 (1968), 999. KLAPPROTH H. J., POUTASSE E. F. and HAZARD J. B.: Renal angiomyolipomas. Arch. Path. 67 (1959), 400. LEMAITRE G., L'HERMINE G., MAILLARD J. P. et LINGNETTE M.: Les angiomyolipomes ou hamartomes du rein. J. Radiol. Electrol. 49 (1968), 315. LOVE L. and FRANK S. J.: Angiographic features of angiomyolipoma of the kidney. Amer. J. Roentgenol. 95 (1965), 406. MCCULLOUGH D. L., SCOTT JR R. and SEYBOLD H. M.: Renal angiomyolipoma (hamartoma). Review of the literature and report of 7 cases. J. Urol. Baltimore 105 (1971), 32. MEANEY T. F.: Errors in angiographic diagnosis of renal masses. Radiology 93 (1969), 361. MEIISEL P. und APITZSCH D. E.: Atlas der Nierenangiographie. Springer-Verlag Berlin, Heidelberg, New York 1978. OWMAN T.: Die Angiographie des renalen Hamartoms. Radiologe 13 (1973), 287. - Renal angiomyolipoma versus renal carcinoma. Is an angiographic differential diagnosis possible? Fortschr. Rontgenstr. 121 (1974), 315. PALMISANO P. J.: Renal hamartoma (angiomyolipoma). Its angiographic appearance and response to intraarterial epinephrine. Radiology 88 (1967), 249. SEABURY J. c., ENSOR R. D. and WOLFE W. G.: Angiornyolipoma of the kidney: A benign tumor demonstrating neovascularisation by arteriography. Report of 2 cases. J. Urol. Baltimore 98 (1969), 562. SILBIGER M. L. and PETERSON C. C.: Renal angiomyolipoma: Its distinctive angiographic characteristics. J. Urol. Baltimore 108 (1971), 511. VIAMONTE JR M., RAVEL R., POLITANO V. and BRIDGES B.: Angiographic findings in a patient with tuberous sclerosis. Amer. J. Roentgenol. 98 (1966), 723. WINDHOLZ F.: Roentgen diagnosis of retroperitoneal lipoma. Amer. J. Roentgenol. 56 (1946), 594.

Downloaded from acr.sagepub.com by guest on July 25, 2015

Advances in the diagnosis of renal angiomyolipoma.

FROM THE KLINIK FUR RADIOLOGIE UND NUKLEARMEDIZIN 1M KLINIKUM STEGLITZ DER FREIEN UNIVERSITAT BERLIN, D-IOOO BERLIN, GERMANY. ADVANCES IN THE DIAGNOS...
768KB Sizes 0 Downloads 0 Views