Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 675

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

ABDOMINAL RADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 3  |  Page : 367-368
CT of diffuse fatty infiltration of renal parenchyma secondary to bilateral giant angiomyolipomas- a case report


Department of Radiodiagnosis & Imaging, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India

Click here for correspondence address and email
 

Keywords: Tuberous sclerosis, bilateral renal angiomyolipomas, cerebral lesions

How to cite this article:
Rawat N S, Negi A, Thukral B B, Saxena N C. CT of diffuse fatty infiltration of renal parenchyma secondary to bilateral giant angiomyolipomas- a case report. Indian J Radiol Imaging 2006;16:367-8

How to cite this URL:
Rawat N S, Negi A, Thukral B B, Saxena N C. CT of diffuse fatty infiltration of renal parenchyma secondary to bilateral giant angiomyolipomas- a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 23];16:367-8. Available from: http://www.ijri.org/text.asp?2006/16/3/367/29019

   Introduction Top


Tuberous sclerosis is an autosomal dominant disorder characterized by a classical clinical triad of epilepsy, mental retardation and adenoma sebaceum. It is associated with multiple organ and skeletal abnormalities [1],[2]. Renal angiomyolipomas are uncommon benign neoplasms that occur in upto 40-80% of tuberous sclerosis patients [2],[3]. Uncommonly they become large in size and have a propensity for spontaneous hemorrhage [4].

We report a rare case of tuberous sclerosis where patient had characteristic cerebral lesions and bilateral renal angiomyolipomas completely replacing renal parenchyma by diffuse fatty infiltration. In addition, patient had a splenic angiomyolipoma.


   Case report Top


A 32 year old female presented to our hospital with dull aching pain since four months & recurrent urinary tract infection since four weeks. On detailed history taking, her family revealed that the patient had episodes of convulsions since childhood for which she took treatment on and off. Physical examination revealed firm masses in bilateral lumbar region occupying almost the whole of abdomen extending from hypochondrium to the iliac region. Laboratory investigations including renal function tests were normal. Chest skiagram was normal. Ultrasound abdomen revealed huge renal masses extending from the hypochondrium to the iliac fossae. These masses were markedly echogenic with no evidence of normal renal parenchyma noted within them. CT scan of abdomen showed bilateral massive renal masses occupying the whole of abdomen and pelvis and causing anterior displacement of bowel loops. Bilateral masses showed low attenuation value suggestive of predominant fat component completely replacing the renal parenchyma. [Figure - 1]

Right-sided mass measured 29 X 16 X 9 cms. in size Left-sided mass measured 27 X 15 X 8 cms. in size

Contrast enhanced scans showed patchy & heterogeneous enhancement in the soft tissue & vascular component of the mass lesion [Figure - 2]. Findings were diagnostic of angiomyolipomas. Normal excretion of contrast media was seen on both sides. Splenomegaly was noted and a well-defined rounded low density lesion with fat and hemorrhagic attenuation value was seen within it. The lesion showed mild enhancement along the periphery [Figure - 3].Possibility of chronic hematoma caused by repeated bleeding from a small angiomyolipoma in spleen was considered. CT scan of brain showed multiple small-calcified subependymal and periventricular nodules [Figure - 4]. As the patient was unwilling for surgery, she was put on conservative management and was advised to come for regular clinical follow up.


   Discussion Top


Tuberous sclerosis is an autosomal dominant disorder often associated with chromosome 9 abnormalities. It is characterized by a classical clinical triad of epilepsy, mental retardation and adenoma sebaceum [1],[2]. However due to incomplete penetrance, symptomatology may range from isolated organ involvement to involvement of multiple organs with hamartomatous lesions particularly in brain, skin, retina, heart & kidneys [1],[2].

Hamartomatous lesions in the brain; typically in the form of subependymal, periventricular calcified nodules are present in more than 50 % of cases [2].Renal involvement occurs in 40-80 % of patients [2][3][4][5]. Renal angiomyolipomas are uncommon benign neoplasms that are composed of mature or immature fat, thick walled blood vessels & smooth muscle elements in varying proportions [2],[4][5][6][7]. They compress and distort the renal parenchyma and collecting system but are not usually invasive [3]. Angiomyolipomas may affect the perirenal fat and local lymphatic vessels and the presence of tumors in extrarenal sites is considered multicentric and not metastatic. As for clinical manifestations, the main symptom is abdominal pain. Other signs and symptoms are palpable mass, hemorrhage, hematuria, nausea and vomiting, systemic arterial hypertension, anemia, fever, shock, renal failure and urinary infection [5]. On ultrasound, the echogenicity of angiomyolipomas is similar to that of renal sinus fat, interspersed with some less echogenic areas. Correlation of ultrasound with histopathology suggests that the echogenic areas correspond primarily to fatty portions of angiomyolipoma, the less echogenic areas to myomatous or vascular elements or to focal hemorrhage while the echo poor lesions are due to hemorrhage [3]. Currently CT is unusually diagnostic due to the attenuation coefficient -10 to -80 HU produced by fatty component of tumor [5]. If fat is depicted at CT, histological proof is not necessary for diagnosis of angiomyolipoma [3],[6]. Since with ultrasound and CT scan there is an improvement in the preoperative diagnosis of angiomyolipoma, percutaneous needle biopsy and surgery for diagnosis should be avoided in asymptomatic individuals for the risk of massive hemorrhage [3]. The current trend is to treat asymptomatic patients conservatively with regular clinical and radiological follow up and to advise nephron sparing surgery and /or intraarterial embolization for those patients with pain, hemorrhage, complex lesions and enlarging tumors [4],[5].

Splenic angiomyolipomas are a very rare association of tuberous sclerosis with only a few isolated case reports in the literature. Similar low density lesions on CT scan with mild enhancement along the periphery can be seen in chronic hematoma caused by repeated bleeding from a small angiomyolipoma in spleen [8]. Although isolated case reports of giant renal angiomyolipomas are seen in literature, but diffuse fatty infiltration completely replacing the renal parenchyma in massively enlarged kidneys in association with a splenic lesion is quite rare [3],[4],[5],[7]

 
   References Top

1.Evans JC, Curtis J. The radiological appearances of tuberous sclerosis. The British Journal of Radiology 2000; 73: 91-98.  Back to cited text no. 1    
2.Arslan A, Ciftci E, Cetin A, Selcuk H, Demirci A. Tuberous Sclerosis: Ultrasound, CT and MRI features of two cases with multiple organ involvement. Australasian Radiology 1998; 42: 379-382.  Back to cited text no. 2  [PUBMED]  
3.Jackson DMA, Collins CD, Cosgrove DO. Case Report: Diffuse fatty infiltration of the renal parenchyma secondary to bilateral angiomyolipomas- features on ultrasound & computed tomography. British Journal of Radiology 1995; 68: 318-320.  Back to cited text no. 3    
4.Ashebu S, Dahniya M, Elshebiny Y, Varro J and Alkhawari H. Giant bleeding renal angiomyolipoma: Diagnosis & Management. Australasian Radiology 2002; 46: 115-118.  Back to cited text no. 4    
5.Schneider-Monteiro ED, Lucon AM, FigueiredoAA, Rodrigues Junior AJ, Arap S. Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis. Rev Hosp Clin Fac Med Sao Paulo. 2003 Mar-Apr; 58(2):103-8. Epub 2003 Jun 25.  Back to cited text no. 5    
6.Kropp B, Dabagia M. Scott JW, Lingeman JE. Percutaneous nephrolithotomy directly through an angiomyolipoma. Urology 1994 Dec; 44[6]: 915-917.  Back to cited text no. 6    
7.Katz DS, Poster RB. Massive renal angiomyolipoma in tuberous sclerosis. Clinical Imaging 1997; 21:200-202.  Back to cited text no. 7    
8.Asayama Y, Fukuya T, Honda H, Kaneko K, Kurolwa T, Yoshimitsu K, Irie H, Shinokuma J. Chronic expanding hematoma of the spleen caused by angiomyolipoma in a patient with tuberous sclerosis. Abdominal Imaging 1998; 23: 527-530.  Back to cited text no. 8    

Top
Correspondence Address:
N S Rawat
25 - B, Pocket A - 10, Kohinoor Apartments, Kalkaji Extension, New Delhi - 110019
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.29019

Rights and Permissions


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Introduction
    Case report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed3810    
    Printed94    
    Emailed0    
    PDF Downloaded262    
    Comments [Add]    

Recommend this journal