Computerizrd Radial. Vol. 6. pp. 287 to 293, 1982 0730-4862/82/050287-07$03.00/O Printed in the U.S.A. All rights reserved Copyright 0 1982 Pergamon Press Ltd

TUBEROUS SCLEROSIS EVALUATED BY COMPUTERIZED TOMOGRAPHY

HARRIS NEWMARK III, D. G. BHAGWANANI, U. S. RISHI, WILBUR S. MELLON JR, MOHAMED HAFEEZ and NAVAL KANT

Department of Radiology, The Western Pennsylvania Hospital, 4800 Friendship Avenue, Pittsburgh, PA 15224, U.S.A.

(Received 5 October 1981; received for publication 22 February 1982)

Abstract-The authors present a case of tuberous sclerosis that shows the typical intracranial and renal changes which were seen on computerized tomography. We know of no other similar report with both abnormalities shown. These findings include intracranial calcifications, especially subependymal, and mul- tiple heterogeneous renal masses. The latter, angiomyolipomas, are contrast enhancing densities with some low attenuation densities equivalent to fat. We feel that in the appropriate clinical setting computerized tomography can be diagnostic of this affliction.

Tuberous sclerosis Hamartoma of the kidney Computerized tomography

Angiomyolipoma Intracranial calcification

INTRODUCTION

The authors have recently encountered a patient with tuberous sclerosis which demonstrated the typical clinical and radiographic findings. The computerized tomographic scan illustrated intracra- nial calcification and bilateral renal hamartomas (angiomyolipomas). The interesting aspects of the case are presented as well as the CT images. Additional CT scans of angiomyolipomas are included.

The CT scanner used was a G.E. 7800 and the studies included both non-enhanced and enhanced scans of the head and abdomen.

CASE REPORTS

The patient wasa 52-yr old black female who complained of progressive right abdominal pain for 2 weeks along with tiredness and weakness. The night before admission she felt as if she would “pass-out”. Her past history was consistent with tuberous sclerosis with sebaceous adenomas in a malar distribution. On physical examination a large right abdominal mass was present. Her hemo- globin was 5.7 mg% and the BUN and creatinine were mildly elevated. The hemoglobin rose to 10 mg% after 6 units of packed cells were transfused. A skull film, I.V.P. and CT scans of the brain and abdomen were performed and showed intracranial calcification in the subependymal region and angiomyolipomas of both kidneys (Figs 1 and 5). A glucoheptonate scan showed very poor function bilaterally with the right somewhat more involved. A bilateral renal arteriogram revealed bilateral renal masses with neovascularity and aneurysmal changes with the left kidney somewhat more spared (Fig. 6). An hepatic arteriogram showed additional angiomyolipomas. A biopsy of one of the facial lesions disclosed adenoma consistent with tuberous sclerosis.

The authors feel this is the first definite case of tuberous sclerosis presented that shows the typical intracranial and renal characteristics seen on CT. Numerous isolated intracranial cases have been presented Cl] and renal angiomyolipomas have been demonstrated on CT [24], but never both together in the same patient. One manuscript [2] shows the computerized appearance of three angiomyolipomas, but there was no definite proof that they occurred in patients with tuberous sclerosis, only a suggestion that bilateral angiomyolipomas might be a forme fruste of this entity. Another paper mainly evaluates their ultrasound characteristics [3].

The differential diagnosis of bilateral heterogeneous renal masses with contrast enhancement should include: renal cell carcinoma, abscess, metastases, and angiomyolipoma. The only one that

287

288 HARRIS NEWMARK III et al.

Fig. 1. A lateral view of the skull shows intracranial calcifications.

Fig. 2. Both kidneys are enlarged and show distortion of the collecting system by the bilateral hamartomas.

Tuberous sclerosis evaluated by CT 289

Fig. 3. A CT scan at the level of the third ventricle shows intracranial calcification in a periventricular location.

should show a significant negative density (fatty density) is angiomyolipoma. It is felt that fatty necrosis of a tumor and liposarcoma should not be as negative in Hounsfelt units.

Classically, tuberous sclerosis consists of the triad: adenoma sebaceum, epileptic seizures, and mental retardation. In addition, paraventricular calcifications with predisposition of the tubers to undergo gliomatous change, phacoma, cystic or sclerotic lesion’of the phalanges, interstitial lung disease with slight increased chance of a pneumothorax and angiomyolipomas of the kidneys, heart, liver and pancreas are noted [l, 51. CT of the head is helpful in demonstrating intracranial calcifica- tions, ventricular dilatation and gliomatous change of a nodule. CT of the abdomen will clearly illustrate the hamartoma and can be diagnostic when it demonstrates the presence of a definite fatty density in a heterogeneous, contrast enhancing renal mass. Hemangiomas and renal cell carcinoma

Fig. 4. This CT body scan shows a large heterogeneous lesion of the right kidney.

290 HARRIS NEWMARK III et al.

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Fig. 5. At a slightly different level, this CT scan shows an addditional heterogeneous lesion of the left kidney.

ecially if there is fatty necrosis) may appear similar but the fatty density will not be as nega renal cell carcinoma. Thus, surgery may be avoided or a subtotal nephrectomy may replal

.1 nephrectomy for this benign lesion. ‘uberous sclerosis is usually inherited as an autosomal dominant disease with varied expres,

Seizures occur in more than 90% of the patients and mental retardation in 60%. By dhood, adenoma sebaceum develops in the malar distribution in 80% of the cases. ngiomyolipomas may present in two distinct groups: one in predominantly middle-age fern rout tuberous sclerosis and the other in younger individuals, with equal sex distribution,

kive ce a

sion late

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Fig. 6. A left selective renal angiogram shows neovascularity and pseudoaneurysms of the left kidney, consistent with an angiomyolipoma.

Tuberous sclerosis evaluated by CT 291

(a)

(b)

Fig. 7. (a) and (b) Two CT scan sections which show angiomyolipomas of both kidneys. This occurred in a patient without tuberous sclerosis.

having the clinical and pathological manifestations of tuberous sclerosis [S]. Angiomyolipomas contain smooth muscle, blood vessels and mature fatty cells and are considered hamartomas. There are varying percentages of the different cell types in each particular case. Their growth can be extrarenal or intrarenal, where there is displacement of calyces and replacement of parenchyma. Two additional cases are presented that show angiomyolipomas in patients without tuberous scler- osis (Figs 7 and 8).

Thus, a CT scan often can be diagnostic of an angiomyolipoma and often suggests tuberous sclerosis if multiple hamartomas are found. One should also make such a diagnosis if intracranial calcifications are seen in a patient who has an angiomyolipoma.

292 HARRIS NEWMARK III et al.

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Fig. 8. (a) and (b) Two CT scans show angiomyolipomas that involve both the right kidney and liver in the same patient.

SUMMARY The authors present a case of tuberous sclerosis that on CT demonstrates the typical intract

calcifications as well as the typical renal hamartomas. Depending on the clinical characteristic CT findings, the definitive diagnosis can be made on CT. The differential diagnosis is discussec feel this is the first case presented that shows both of these two features in a single patient on

Acknowfedgement-We appreciate the excellent secretarial assistance by Sheila A. Faust.

REFERENCES 1. E. P. Barmeri, H. I. Price and A. Danziger, The value of computed tomography in tuberous sclerosis, S. Ajr. Med.

588 (1980).

ra

s. <

J.

inial and We

IT.

57.

Tuberous sclerosis evaluated by CT 293

2. G. C. Hansen, R. B. Hoffman, W. F. Sample and R. Becker, Computed tomography diagnosis of renal angiomyolipoma, Radiology 128, 789 (1978).

3. D. S. Hartman, S. M. Stanford, A. C. Friedman, C. J. Davis Jr, J. E. Madewell J. L. Sherman, Angiomyolipoma: ultrasonic-pathologic correlation, Radiology 139, 451-458 (1981).

4. A. C. Friedman, D. S. Hartman, J. Sherman, E. M. Lautin and M. Goldman, Computed tomography of abdominal fatty masses, Radiology 139, 415-429 (1981).

5. J. L. Emmett and D. M. Witten, Clinical Urography, 3rd edn. Saunders, Philadelphia, PA (1971). 6. A. R. Kagan, R. J. Steckel, H. Kangaloo and J. Pagari, Diagnostic oncology case study; flank pain and hematuria in a

child, Am. J. Roentg. p. 597 (1981).

About the Author-HARRrs NEWMARK III received his M.D. degree from the Cniversity of Southern California in 1968 and completed his residency in diagnostic radiology in 1975, at the same institution. A neuroradiology fellowship followed. He is currently head of special procedures and neuroradiology at the Western Pennsylvania Hospital. He has published numerous articles on CT and neuroradiology.

About the Author-DRUPADI G. BHAGWANANI received her M.B.B.S. degree at SM.!% Medical College in Jaiour. India. She received her oost-graduate diploma in OBIGYNE. Bombav, India. Dr Bhagwanani coipleted her Diagnostic Radiology training at Wkst Penn Hospital, Pittsburgh, Pennsylvania in 1971. She received a diploma from the American Board of Radiology in 1972. Dr Bhagwanani has been a staff radiologist at West Penn Hospital since 1971. In 1974 Dr Bhagwanani became in charge of the Residency Training Program. Her special interests are CAT scanning, mammography and pediatric radiology.

About the Author-UsHA U. RISHI received her M.B.B.S. degree at S.M.S. Medical College, Jaipur, India. She received her post-graduate diploma in TB in Bombay, India. Dr Rishi completed her Diagnostic Radiology Residency in 1968 at the Western Pennsylvania Hospital, Pittsburgh, Pennsylvania. She was a fellow for one year at the University of Pittsburgh Radiology Department, 1968-1969 and received a diploma from the American Board of Radiology in 1969. Dr Rishi has been at West Penn Hospital since 1970 as a staff radiologist. Her special interests are CAT scanning, mammography and pediatric radiology.

About the Author-WILBUR S. MELLON JR received his M.D. from the University of Pittsburgh Medical School in 1948 and completed a radiology residency at the Western Pennsylvania Hospital. He then served for 2 yr in the US. Army Medical Corp. as a radiologist. He returned to the staff of the Western Pennsyl- vania Hospital where he has been Chairman of the Radiology Department since 1959. He is a Clinical Assistant Professor of Radiology, School of Medicine, University of Pittsburgh and is a Diplomat of the American Board of Radiology.

About the Author-MOHAMMAD HAFEEZ received his M.D. from King Edward Medical School (Lahore, Pakistan) in 1969. He completed his internship and residency training in Diagnostic Radiology at Wayne State University School of Medicine (Detroit, Michigan) from 19741978. He is currently attending staff in the Department of Radiology in Western Pennsylvania Hospital (Pittsburgh, Pennsylvania). He is Diplomat of American Board of Radiology. His practice includes angiography, interventional procedures and CT scan.

About the Author-NAVAL KANT received M.B.B.S. degree from the University of Rangoon, Faculty of Medicine, Rangoon, Burma in 1962. He practiced Medicine until he migrated to U.S.A. in 1971. He completed his residency training in Radiology in 1976 and was appointed Assistant Professor in Clinical Radiology at the University of Pittsburgh. He is board certified in his specialty and is currently practicing Radiology. focusing on Special Procedures, at West Penn Hospital, Pittsburgh, Pennsylvania.