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DOI:10.2214/AJR.04.1906
AJR 2005; 185:1085-1086
© American Roentgen Ray Society

Bilateral Massive Renal Angiomyolipomatosis in Tuberous Sclerosis

Howard Liu, Kenneth Cooke and David Frager

St. Luke's-Roosevelt Hospital Center New York, NY 10019

Renal angiomyolipoma is arare benign neoplasm composed of varying amounts of mature adipose tissue, smooth muscle, and blood vessels. Angiomyolipomas, particularly whent multiple or bilateral, suggest the diagnosis of tuberous sclerosis. We describe the case of a young woman with massive bilateral renal angiomyolipomas and tuberous sclerosis.



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  		Fig. 2A —27-year-old woman with bilateral renal angiomyolipoma in tuberous sclerosis. Axial (A) and coronal (B) unenhanced CT images show massive bilateral renal angiomyolipoma replacing both kidneys.

 



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  		Fig. 2B —27-year-old woman with bilateral renal angiomyolipoma in tuberous sclerosis. Axial (A) and coronal (B) unenhanced CT images show massive bilateral renal angiomyolipoma replacing both kidneys.

 



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  		Fig. 2C —27-year-old woman with bilateral renal angiomyolipoma in tuberous sclerosis. Axial unenhanced CT scan of chest shows multiple lung cysts suggestive of lymphangioleiomyomatosis.

 



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  		Fig. 2D —27-year-old woman with bilateral renal angiomyolipoma in tuberous sclerosis. Axial unenhanced CT scan of brain shows numerous calcified subependymal nodules.

 
A 27-year-old woman presented to our institution complaining of vague abdominal pain and distention for several months. Clinical examination revealed firm masses bilaterally in the flanks occupying almost the entire abdomen. Her face showed plaquelike lesions in the forehead and nasolabial folds consistent with adenoma sebaceum. On questioning, the patient revealed a history of seizures since childhood. Serum chemistry showed a slightly elevated creatinine level of 1.4 mg/dL. CT of the abdomen and pelvis revealed huge bilateral masses consisting mostly of fat and occupying almost the entire abdomen and replacing both kidneys (Figs. 2A and 2B). The masses had displaced the intestines and mesentery anteriorly. The right mass measured 25 x 17.5 x 12.5 cm, and the left measured 30 x 19 x13.6 cm. CT of the chest revealed multiple small air cysts scattered throughout the lungs (Fig. 2C), and CT of the brain showed scattered calcified densities along both lateral ventricles consistent with subependymal nodules or tubers (Fig. 2D).

Because of the presence of bilateral angiomyolipomas, subependymal tubers, lymphangioleiomatosis of the lungs, and adenoma sebaceum of the face, the patient was diagnosed as having a case of tuberous sclerosis with bilateral renal angiomyolipomatosis.

The natural history of renal angiomyolipoma is unknown. Patients who have renal angiomyolipomas with tuberous sclerosis are distinctly different from those without tuberous sclerosis. Patients with tuberous sclerosis present at a younger age, are more likely to be symptomatic, and have large bilateral tumors that are more likely to grow and therefore more frequently require surgery. In the series by Steiner et al. [1], the average size of angiomyolipoma in patients with tuberous sclerosis is 9.6 ± 4.8 cm, and in those without tuberous sclerosis, is 4.1 ± 3.4 cm. Angiomyolipoma may grow to be large and bulky and extend into the perirenal space, which sometimes makes it difficult to differentiate from perirenal liposarcoma. However, perirenal liposarcoma is unilateral and usually will not invade the kidney because it arises from retroperitoneal fat. In comparison, renal angiomyolipoma arises from the renal parenchyma and involves the kidney itself [2]. Here, we have presented a case of massive bilateral renal angiomyolipomatosis filling the entire abdomen and replacing the renal parenchyma bilaterally.

To our knowledge, only one published case report of such massive bilateral angiomyolipoma has been presented in the literature [3]. Usually patients will present much earlier, before the angiomyolipoma reaches the size seen in our patient, with symptoms such as hemorrhage, flank pain, renal failure, anemia, or palpable mass. This case illustrates the association of renal angiomyolipoma in a patient with tuberous sclerosis. Renal angiomyolipomas may grow substantially. Especially in cases of bilateral large renal angiomyolipomas, one should strongly consider the diagnosis of tuberous sclerosis.


References
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 References
 

  1. Steiner MS, Stanford GM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol1993; 150:1762 -1786
  2. Israel GM, Bosniak MA, Slywotzky CM, Rosen RJ. CT differentiation of large exophytic renal angiomyolipomas and perirenal liposarcoma. AJR 2002; 179:769 -773[Abstract/Free Full Text]
  3. Khan AS, Bakhshi GD, Siddiqui AQ, et al. Massive bilateral renal angiomyolipomatosis in tuberous sclerosis. BHJ2003; 45:477 -480

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