Meta analysis of the relationship between tuberous sclerosis complex and renal cell carcinoma

Abstract

The association of angiomyolipoma with tuberous sclerosis complex (TSC) patients is well recognised. Some literature has suggested that patients with TSC are at increased risk of renal neoplasm, particularly since angiomyolipomas can be locally invasive and demonstrate tumor like neovascularity on angiography. In an attempt to place in context one case of metastatic renal cell carcinoma in a patient with TSC a meta analysis of the literature was performed. One pediatric patient with TSC and metastatic renal cell carcinoma was evaluated. Reviews and case reports of TSC and renal cell carcinoma and related imaging findings were identified by searching the MEDLINE database, the `bibliography of reviews' in Index Medicus, personal files and the reference lists from all identified reviews. Individual cases suitable for review in the available literature from 1922 through 1993 comprised the data base. The data base was analysed with the method of proportions and unpaired two-tailed Student's t-test for different populations. A cumulative summary of the literature amassed 107 additional cases from 62 reports over 71 years. Our case is the 17th case, to our knowledge, of renal cell carcinoma in TSC. Analysis of the distribution of characteristics and incidence of; renal cysts, renal cell carcinoma, and lymph node involvement demonstrate no increased risk of renal cell carcinoma in patients with TSC ( P=NS). Though anecdotal evidence has suggested a relationship between TSC and renal cell carcinoma, meta analysis of cases of coincident TSC renal complex, cystic changes, angiomyolipoma and renal cell carcinoma implies an identical, though small, risk of malignancy in TSC patients as compared with the normal population.