Composite renal cell carcinoma and angiomyolipoma in a patient with Tuberous sclerosis: A diagnostic dilemma

Authors

  • Aanchal Kakkar Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
  • Archana George Vallonthaiel Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
  • Mehar C. Sharma Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
  • Girdhar Bora Department of Urology, All India Institute of Medical Sciences, New Delhi, India
  • Ananya Panda Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India
  • Amlesh Seth Department of Urology, All India Institute of Medical Sciences, New Delhi, India

DOI:

https://doi.org/10.5489/cuaj.2532

Keywords:

renal cell carcinoma, angiomyolipoma, Tuberous sclerosis, collision tumor, composite tumor

Abstract

Tuberous sclerosis (TS) is an autosomal dominant disorder associated with various renal pathologies, including angiomyolipoma (AML). Renal cell carcinoma (RCC) with concomitant AML is rare. We report a case of composite RCC and AML in a young male with TS. This 25-year-old male presented with an abdominal mass. The abdominal computed tomography scan revealed RCC in the left kidney and AML in right kidney. A left radical nephrectomy was performed. Microscopy showed a tumour composed of closely intermingled RCC and AML components. Immunohistochemistry was performed for confirmation. A diagnosis of composite tumour with clear cell RCC and AML was made. Though the coexistence of RCC with AML is rare, and the composite variant is even rarer, one must be aware of this entity to prevent misdiagnosis as well as upstaging of RCC, and also to avoid missing areas of RCC within a large AML, especially in TS patients.

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Published

2015-07-17

How to Cite

Kakkar, A., Vallonthaiel, A. G., Sharma, M. C., Bora, G., Panda, A., & Seth, A. (2015). Composite renal cell carcinoma and angiomyolipoma in a patient with Tuberous sclerosis: A diagnostic dilemma. Canadian Urological Association Journal, 9(7-8), E507–10. https://doi.org/10.5489/cuaj.2532

Issue

Section

Case Report