META ANALYSIS OF THE RELATIONSHIP BETWEEN TUBEROUS SCLEROSIS COMPLEX AND RENAL-CELL CARCINOMA

The association of angiomyolipoma with tuberous sclerosis complex (TSC ) patients is well recognised. Some literature has suggested that pati ents with TSC are at increased risk of renal neoplasm, particularly si nce angiomyolipomas can be locally invasive and demonstrate tumor like neovascularity on angiography. Tn an attempt to place in context one case of metastatic renal cell carcinoma in a patient with TSC a meta a nalysis of the literature was performed. One pediatric patient with TS C and metastatic renal cell carcinoma was evaluated. Reviews and case reports of TSC and renal cell carcinoma and related imaging findings w ere identified by searching the MEDLINE database, the 'bibliography of reviews' in Index Medicus, personal files and the reference lists fro m all identified reviews. Individual cases suitable for review in the available literature from 1922 through 1993 comprised the data base. T he data base was analysed with the method of proportions and unpaired two-tailed Student's t-test for different populations. A cumulative su mmary of the literature amassed 107 additional cases from 62 reports o ver 71 years. Our case is the 17th case, to our knowledge, of renal ce ll carcinoma in TSC. Analysis of the distribution of characteristics a nd incidence of renal cysts, renal cell carcinoma, and lymph node invo lvement demonstrate no increased risk of renal cell carcinoma in patie nts with TSC (P = NS). Though anecdotal evidence has suggested a relat ionship between TSC and renal cell carcinoma, meta analysis of cases o f coincident TSC renal complex, cystic changes, angiomyolipoma and ren al cell carcinoma implies an identical, though small, risk of malignan cy in TSC patients as compared with the normal population. (C) 1998 El sevier Science Ireland Ltd. All rights reserved.