Stephen Karasick, MD

Richard J. Wechsler, MD

Case 23: Replacement Lipomatosis

of the Kidney1
HISTORY
A 49-year-old man underwent computed tomography (CT)
prior to possible bilateral nephrectomy. He had a history of
end-stage renal disease secondary to a neurogenic bladder with
chronic reflux nephropathy and chronic pyelonephritis. The
patient also had spina bifida, hypertension, hepatitis B, atrial
fibrillation, hypothyroidism, and degenerative joint disease.
He had been receiving hemodialysis for 6 years and has a
ureteroileostomy.
A digital abdominal radiograph (Fig 1) was obtained prior to
abdominal pelvic CT. CT scans through the top of the kidneys
at the level of the celiac axis (Fig 2) and through the lower pole
of the left kidney (Fig 3) are shown.

IMAGING FINDINGS
A digital abdominal radiograph (Fig 1) obtained prior to CT
showed markedly enlarged kidneys with multiple renal calculi.
A large, smoothly lobulated 9 3 4-cm calculus was noted
overlying the right renal pelvis and ureter. A transverse CT scan
through the upper kidneys (Fig 2) revealed extreme parenchymal atrophy with marked fatty proliferation within the renal
sinus, hilus, and perinephric space. A transverse CT scan
through the lower pole of the left kidney (Fig 3) revealed left
renal calculi and a large right staghorn calculus. An ultrasonographic (US) scan, obtained in the left kidney (Fig 4) several
months before the CT scans, revealed enlargement of the

Index terms:
Diagnosis Please
Kidney, calculi, 81.811
Kidney, CT, 81.1211
Kidney, US, 81.1298
Lipoma and lipomatosis, 81.3119
Nephritis, 81.2125, 81.213
Radiology 2000; 215:754756
1

From the Department of Radiology, Thomas Jefferson University

Hospital, 111 S 11th St, Ste 3390, Philadelphia, PA 19107. Received
December 21, 1998; revision requested February 9, 1999; revision
received March 3; accepted July 30. Address correspondence to S.K.
(e-mail: Stephen.Karasick@mail.tju.edu).
r RSNA, 2000

754

kidney with preservation of its reniform shape. There was a

highly echogenic appearance to the renal sinus consistent with
fat that extended to the periphery and occupied most of the
renal tissue. A 1.5-cm stone was present in the lower pole of the
left kidney (Fig 5).

DISCUSSION
Replacement lipomatosis of the kidney, also known as replacement fibrolipomatosis, is an advanced form of renal sinus
lipomatosis that usually occurs unilaterally. A varying amount
of fat and fibrous tissue is always present within the renal sinus,
which becomes more prominent with aging, obesity, and use of
exogenous steroids. Replacement fibrolipomatosis represents
the extreme form of renal sinus lipomatosis in which infection,
long-term hydronephrosis, and calculi are associated with
severe renal parenchymal atrophy (18). Renal calculous disease associated with inflammatory changes is found in more
than 70% of cases (8). Clinical symptoms, including urinary
tract infections, fever, and flank pain, usually result from the
associated inflammatory and calculous disease (2).
Pathologically, the kidney usually is enlarged and has a gross
fibrofatty appearance. The renal cortex is extremely atrophied,
with varying degrees of hydronephrosis or pyonephrosis, as
well as having acute and chronic pyelonephritic changes. The
reniform shape of the kidney is maintained. There is marked
proliferation of hyperplastic fat in the renal sinus, with extremely large fat cells that do not permeate the renal parenchyma but merely develop adjacent to it as it atrophies (3,4).
This process is distinct from that seen with lipomas, which are
found within the parenchyma.
The abdominal radiograph characteristically demonstrated a
staghorn calculus with an enlarged renal outline (Fig 1).
Excretory urography demonstrated a poorly functioning or
nonfunctioning kidney. At US, there was expansion of the
hyperechoic mass that represented lipomatous tissue in the
central sinus (Fig 4). A central high-intensity echo with acoustic shadowing represented the staghorn or other calculus (Fig
5). It may be difficult to see the hypoechoic rim of the residual
parenchyma (8). Although US may show highly suggestive
findings, CT is the most accurate method of demonstrating the
distinctive features of replacement lipomatosis. The staghorn
calculus and the atrophied renal parenchyma were depicted
easily (Figs 2, 3). The abundant fatty tissue centrally has the
characteristic attenuation of fat (510). The characteristic distribution of fat within the renal sinus and perinephric space is
unique to replacement lipomatosis. If surgery is indicated, CT

Figure 3. Transverse contrast-enhanced abdominal CT scan obtained through the lower pole of the left kidney reveals scattered left
renal calculi and a large right staghorn calculus (*).

Figure 1. Anteroposterior digital abdominal radiograph obtained

prior to abdominal CT reveals enlarged kidneys with scattered calculi
(arrows) and right staghorn calculus (*).

Figure 4. Transverse US scan obtained through

the upper pole of the left kidney reveals the
highly echogenic appearance of the renal sinus
(calipers) with fat that extends to the periphery
and replaces most of the renal tissue. The
kidney, however, has preserved its reniform
shape.

Figure 2. Transverse contrast materialenhanced abdominal CT

scan obtained through the upper kidneys reveals extreme renal
parenchymal atrophy with only a thin rim (arrows) of renal cortex
remaining. The renal sinus and hilus have been replaced with fat that
extends into the perinephric space. The rounded masses of soft-tissue
attenuation adjacent to the right kidney represent exophytic renal
cysts.

can be valuable in the preoperative planning by demonstrating

the absence or presence of abscess and fistula formation.
The major differential diagnosis in the presence of longstanding inflammation and calculous obstruction is xanthogranulomatous pyelonephritis. Lipid-laden macrophages actually infiltrate the renal parenchyma in contrast to replacement
Volume 215 Number 3

Figure 5. Transverse US scan obtained through

the lower pole of the left kidney revels a 1.5-cm
stone (arrow) with shadowing beneath.

lipomatosis in which the fat cells remain outside of the

atrophied renal parenchyma. The features at US and CT are,
therefore, different. At US, with xanthogranulomatous pyelonephritis, there are hypoechoic areas that represent purulent
material, as well as medium-amplitude echoes that correspond
to the fibrofatty and/or necrotic debris (8). CT shows hydroneReplacement Lipomatosis of the Kidney 755

phrosis or pyonephrosis along with xanthogranulomatous

tissue, which typically has attenuation values close to that of
water (215 to 115 HU).
Xanthogranulomatous pyelonephritis and replacement fibrolipomatosis may coexist (11). Replacement fibrolipomatosis
may be tumefactive, in which case other focal fatty lesions such
as lipoma, angiomyolipoma, and liposarcoma must be considered. The absence of parenchymal atrophy and staghorn calculus are additional clues for excluding these entities (6).
References
1. Young HH. Lipomatosis or destructive fat replacement of the renal
cortex. J Urol 1933; 29:631644.
2. Gildenhorn HL. Renal replacement lipomatosis: review and case
report. JAMA 1962; 181:994996.
3. Ambos MA, Bosniak MA, Gordon R, Madayag MA. Replacement
lipomatosis of the kidney. AJR Am J Roentgenol 1978; 130:1087
1091.

4.
5.
6.
7.
8.
9.
10.
11.

Hurwitz RS, Benjamin JA, Cooper JF. Excessive proliferation of

peripelvic fat of the kidney. Urology 1978; 11:448456.
Honda H, McGuire CW, Barloon TJ, Hashimoto K. Replacement
lipomatosis of the kidney: CT features. J Comput Assist Tomogr
1990; 14:229231.
Kullendorff B, Nyman V, Aspelin P. Computed tomography in
renal replacement lipomatosis. Acta Radiol 1987; 28:447450.
Thierman D, Haaga JR, Anton P, LiPuma JP. Renal replacement
lipomatosis. J Comput Assist Tomogr 1983; 7:341343.
Subramanyam BR, Bosniak MA, Horii SC, Megibow AJ, Balthazar
EJ. Replacement lipomatosis of the kidney: diagnosis by computed
tomography and sonography. Radiology 1983; 148:791792.
Nicholson DA. Case report: replacement lipomatosis of the kidneyunusual CT features. Clin Radiol 1992; 45:4243.
Amis ES, Cronan JJ. The renal sinus: an imaging review and
proposed nomenclature for sinus cysts. J Urol 1988; 139:1151
1159.
Acunas B, Acunas G, Rozanes I, Buyokbabani N, Gokmen E.
Coexistent xanthogranulomatous pyelonephritis and massive replacement lipomatosis of the kidney: CT diagnosis. Urol Radiol
1990; 12:8890.

Our congratulations to the 34 individuals who submitted the most likely diagnosis (replacement lipomatosis of the kidney) for
Diagnosis Please, Case 23. The names and locations of the individuals, as submitted, are as follows:
Marco A. Amendola, MD, Miami, Fla
Yasutaka Baba, Kagoshima, Japan
Edward L. Baker, MD, San Francisco, Calif
Ken Baliga, Rockford, Ill
Marc P. Banner, MD, Voorhees, NJ
Etta Barracciu, Cagliari, Italy
Giuseppe Brancatelli, MD, Palermo, Italy
Eric L. Bressler, Minnetonka, Minn
Christophe J. Chagnaud, MD, Marseille, France
Kemal Demir, MD, Istanbul, Turkey
Olivier Dourthe, MD, Sophia Antipolis, France
Seyed A. Emamian, MD, PhD, Washington, DC
Howard T. Heller, MD, Garden City, NY
Ryuji Katada, MD, Sapporo, Japan
Richard A. Leder, MD, Durham, NC
N. B. S. Mani, MD, Chandigarh, India
Antonio Medina Bentez, Granada, Spain

756 Radiology June 2000

Julio Mendez-Uriburu, Tucuman, Argentina

Manabu Minami, MD, Tokyo, Japan
Robert Mindelzun, MD, Palo Alto, Calif
Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina
Dr. Miguel Eduardo Nazar, Capital Federal, Argentina
Carlo L. E. Petralli, Switzerland
James Ravenel, MD, Syracuse, NY
Enrique Remartinez Escobar, MD, Melilla, Spain
Pierre-Jean Sauvage, MD, Macon, France
Matt Shapiro, MD, Lowell, Mass
Taro Shimono, MD, Kyoto, Japan
Paolo Siotto, MD, Cagliari, Italy
Toshiaki Takeda, Tokyo, Japan
Douglas L. Teich, MD, Brookline, Mass
D. Dean Thornton, MD, Kirkwood, Mo
Kay Vilanova, MD, Girona, Spain
Joe Yut, Olathe, Kan

Karasick and Wechsler