(Radiographics. 1999;19:1388-1389.)
© RSNA, 1999
Pediatric Case of the Day1
Janice W. Allison, MD ,
Charles A. James, MD and
Maria S. Figarola, MD
1 From the Department of Radiology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, 800 Marshall St, Little Rock, AR 72202 (J.W.A., C.A.J.), and the Department of Radiology, University of South Alabama Medical Center, Mobile, AL 36617 (M.S.F.). From the 1998 RSNA scientific assembly. Received April 5, 1999; revision requested May 4 and received June 9; accepted June 9. Address reprint requests to J.W.A.
Index Terms: Kidney neoplasms, 81.324 • Neoplasms, in infants and children, 81.324 • Sclerosis, tuberous, **.18322
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HISTORY
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An 11-year-old girl presented with right flank pain and anemia. The patient's medical history was significant for a seizure disorder and mental retardation. Computed tomography (CT) and renal arteriography were performed. Follow-up CT was performed several weeks later.
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FINDINGS
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Initial axial CT through the midportion (Fig 1a) and lower pole (Fig 1b) of the right kidney revealed a large intrarenal mass and perirenal hematoma. The mass was inhomogeneous but contained no areas of fat attenuation. Right renal arteriography showed no evidence of aneurysm (Fig 2). Follow-up axial CT through the midportion of the kidney revealed a more clearly defined mass and substantial resolution of the perirenal hemorrhage (Fig 3).
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Figure 1a. (a) Axial CT scan through the midportion of the right kidney obtained immediately after surgery reveals a large intrarenal mass and perirenal hematoma. The mass is inhomogeneous but contains no areas of fat attenuation. (b) Axial CT scan through the lower pole of the right kidney also reveals a large intrarenal mass and surrounding hemorrhage.
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Figure 1b. (a) Axial CT scan through the midportion of the right kidney obtained immediately after surgery reveals a large intrarenal mass and perirenal hematoma. The mass is inhomogeneous but contains no areas of fat attenuation. (b) Axial CT scan through the lower pole of the right kidney also reveals a large intrarenal mass and surrounding hemorrhage.
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Figure 2. Early digital subtraction image from a right renal arteriogram demonstrates no aneurysm that might account for the perirenal hemorrhage (cf Fig 1). A dominant mass is seen in the midportion and lower pole of the kidney; the mass is draped with vessels (arrows) and has tumor vascularity in the medial portion (arrowheads).
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Figure 3. Axial CT scan through the midportion of the right kidney obtained a few weeks later reveals a more clearly defined, inhomogeneous mass with a larger solid component and resolution of much of the surrounding hemorrhage.
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DIAGNOSIS: Renal cell carcinoma in a child with tuberous sclerosis.
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DISCUSSION
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Tuberous sclerosis is an autosomal dominant, multisystem, neurocutaneous disorder. The classic clinical triad consists of seizures, mental retardation, and adenoma sebaceum. Hamartomas occur in various organs and include cortical tubers in the brain, retinal phakomas, skin angiofibromas, rhabdomyomas of the heart, sclerotic lesions in the bones, lymphangiomas in the lungs, and angiomyolipomas and renal cysts in the kidneys (1). Renal lesions are seen in 50% of cases (1). The overall prognosis for tuberous sclerosis is poor, with 75% of patients dying from complications of renal failure by 20 years of age (1).
The renal lesions in tuberous sclerosis are usually asymptomatic initially (1,2), although they can cause flank pain, hematuria (microscopic hematuria was seen at pathologic analysis in this case), and hypertension. Angiomyolipomas are the most common lesion, followed by renal cysts. Angiomyolipomas are composed of abnormal blood vessels, smooth muscle, and mature adipose tissue (2). They can rupture, leading to retroperitoneal hemorrhage and shock (1). Renal cysts are seen alone or in conjunction with angiomyolipomas and are often multiple and bilateral (2).
Renal cell carcinomas occur in adults with tuberous sclerosis but rarely in children. The only radiologic finding that can help distinguish an angiomyolipoma from a renal cell carcinoma is intralesional fat at CT (1–3). Use of unenhanced CT and thin sections may improve detection of this fat due to increased spatial and contrast resolution and decreased susceptibility to partial-volume effects (3). Only a small percentage of angiomyolipomas do not contain fat. Other fat-containing tumors of the kidney include lipomas, which contain large amounts of fat; well-differentiated liposarcomas; and teratomas, which are extremely rare and contain calcium (3). Although renal cell carcinomas may be seen to contain fat at gross pathologic examination, they do not have negative attenuation values at CT (2).
The prevalence of renal cell carcinoma in patients with tuberous sclerosis is unknown. In 1998, Tello et al (4) reported that only 17 cases had been documented in the literature. On the basis of metanalysis, the authors suggested that the prevalence of renal cell carcinoma in patients with tuberous sclerosis is identical to that in the general population, although anecdotal evidence suggests otherwise (4).
This case illustrates a rare renal tumor in a child with tuberous sclerosis. A metastatic work-up revealed no evidence of metastatic disease. The patient underwent radical right nephrectomy and will continue to be followed up with CT.
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Footnotes
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** indicates multiple body systems 
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References
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Robertson FM, Cendron M, Klauber GT, Harris BH. Renal cell carcinoma in association with tuberous sclerosis in children. J Pediatr Surg 1996; 31:729-730.[Medline]
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Stillwell TJ, Gomez MR, Kelalis PP. Renal lesions in tuberous sclerosis. J Urol 1987; 138:477-481.[Medline]
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Bosniak MA, Megibow AJ, Hulnick DH, Horii S, Raghavendra BN. CT diagnosis of renal angiomyolipoma: the importance of detecting small amounts of fat. AJR 1988; 151:497-501.[Abstract/Free Full Text]
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Tello R, Blickman JG, Buonomo C, Herrin J. Meta-analysis of the relationship between tuberous sclerosis complex and renal cell carcinoma. Eur J Radiol 1998; 27:131-138.[Medline]
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HISTORY
FINDINGS
