RESIDENT CASE STUDIES

Week 5 May 19 - May 23, 2003: Case 2

Table of Contents | List of Diagnoses | Case 1 | Case 2 | Case 3 | Case 4

28 year old female with history of tuberous sclerosis, with a large (>20 cm in diameter) retroperitoneal mass

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Questions:

1. The histologic features are most consistent with:
   1. Renal cell carcinoma
   2. Sarcoma with infiltration of fat
   3. Angiomyolipoma 
   4. Aggressive angiomyxoma 
   5. None of the above 

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2. The following is true of the tumor:
   1. It is never associated with pulmonary lymphangioleiomyomatosis (LAM) 
   2. It is almost always associated with tuberous sclerosis 
   3. Involvement of lymph nodes, renal vein or spleen is a sign of malignancy
   4. HMB 45 is positive in 5% of cases
   5. It may undergo malignant transformation, albeit rarely