Kosin Med J.  2013 Dec;28(2):177-182. 10.7180/kmj.2013.28.2.177.

A Tubercus Sclerosis Case Accompanied by Cystic Angiomyolipoma and Chronic Kidney Disease Diagnosed during Treatment for Acute Cerebral Infarction

• Kim H
• Moh I
• Jung DH
• Lee YK
• Woo JY
• Lee Y
• Noh JW

• KMID: 1993578
• DOI: http://doi.org/10.7180/kmj.2013.28.2.177

Abstract

Symptoms of tuberous scelrosis (TS) are mainly related with brain and kidneys. Seizure, mental retardation, other behavioral problems are dominant. A spectrum of renal tumors from benign angiomyolipoma (AML) to polycystic kidney disease, and rarely malignant renal cell carcinoma have been observed. Cystic AML is a rare phenotype of AML. No case of TS with renal cystic AML has been reported in Korea yet. And chronic kidney disease (CKD) in TS has been seldom reported. We experienced a TS case accompanied by renal cystic AML and CKD diagnosed in a 48-year-old female patient who was hospitalized for left side weakness and seizure under the diagnosis of acute cerebral infarction.

Keyword

Angiomyolipoma; Cerebral infarction; Chronic renal failure; Tuberous sclerosis

MeSH Terms

Angiomyolipoma*
Brain
Carcinoma, Renal Cell
Cerebral Infarction*
Diagnosis
Female
Humans
Intellectual Disability
Kidney
Kidney Failure, Chronic
Korea
Middle Aged
Phenotype
Polycystic Kidney Diseases
Renal Insufficiency, Chronic*
Sclerosis*
Seizures
Tuberous Sclerosis

Figure

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  Fig. 1. Genogram. ◎ (patient)

• 

  Fig. 2. (A) Brain CT shows multiple calcifications suggesting calcified tubers in right frontal periventricular area and left frontal subcortical white matter (arrows), (B) FLAIR image of MRI shows multifocal linear and wedge shaped high signal lesions suggesting cortical and subcortical tubers in both frontal cortex and subcortical white matter (arrows).

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  Fig. 3. Cystic angiomyolipoma showing fat component (arrows) in the left kidney in abdominal CT.

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  Fig. 4. (A) T2, (B) T1 fat setting, (C) T1 GRE, (D) T1. About 3.8 cm sized cystic mass with mixed components suggesting cystic angiomyolipoma and multiple cysts are seen (arrows) in MRI examination.

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