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Case report: perinephric angiomyolipomas in tuberous sclerosis
1Department of Diagnostic Radiology, University of Hong Kong, Queen Mary Hospital.
The British Journal of Radiology
|October 1, 1994
Summary
Tuberous sclerosis can cause bilateral large perinephric angiomyolipomas and lung cysts. This case highlights the importance of recognizing these rare manifestations in affected individuals.
Area of Science:
- Nephrology
- Radiology
- Genetics
Background:
- Tuberous sclerosis is a genetic disorder characterized by the development of tumors in various organs.
- Perinephric angiomyolipomas are rare tumors that can occur in patients with tuberous sclerosis.
- Pulmonary involvement, such as cysts, is also a known complication of tuberous sclerosis.
Observation:
- A young Chinese woman with tuberous sclerosis presented with bilateral large perinephric angiomyolipomas.
- Computed tomography (CT) revealed large fatty masses with abnormal blood vessels and muscle strands within Gerota's fascia.
- Multiple foci of involvement were observed in both kidneys.
Findings:
- Histological examination confirmed the diagnosis of perinephric angiomyolipomas.
- The patient later developed bilateral pneumothoraces, requiring surgical intervention.
- High-resolution CT demonstrated extensive thin-walled cysts in both lungs.
Implications:
- This case underscores the potential for significant renal and pulmonary complications in tuberous sclerosis.
- Early diagnosis and management of angiomyolipomas and pneumothoraces are crucial for patient outcomes.
- Further research into the pathogenesis and treatment of these manifestations is warranted.