RENAL ANGIOMYOLIPOMA - REPORT OF 3 CASES WITH REGIONAL LYMPH-NODE INVOLVEMENT AND OR WITH RENAL-CELL CARCINOMA/

Aims and background: Angiomyolipomas (AMLs) are benign hamartoid tumor s which frequently occur in tuberous sclerosis (TS), They may be manif est at different organ sites such as kidneys, lymph nodes, liver and l ung and may be associated with renal cell carcinoma (RCC), The nature of multiple organ involvement in AML (metastasis versus multicentric s ynchronous tumors), the malignant transformation and the relation of A ML to RCC have not been sufficiently clarified, Study design: Three ca ses of renal AMLs in patients with tuberous sclerosis associated with lymphangioleiomyomatosis of the paraaortic lymph nodes and/or with RCC are reported, The concise clinical history of the patients as well as the findings of histology, immunohistochemistry and quantitative DNA analysis are presented, Results: The multicentric form of AML and coin cidence of renal AML and RCC were observed in 2 patients, AML and RCC were found within the same focus in one of the cases, RCCs were either aneuploid or ''near diploid'', whereas one of the multicentric AMLs s howed a discordant DNA ploidy pattern, namely aneuploidy in the kidney and diploidy in the lymph nodes. Conclusions: The presented cases (al l of them underwent periaortic lymphadenectomy) suggest that lymph nod e involvement in renal AML may be more frequent than expected (1-2% of all AMLs) on the basis of the few reported cases. The discordant DNA ploidy (renal versus lymph node lesions) observed in one of the cases with multicentric AML implies synchronous tumor growth at different si tes rather than metastatic disease, The intimate coexistance of RCC an d AML (RCC revealed by immunohistochemistry within a larger mass of re nal AML) may indicate that malignant transformation of an AML should o nly be accepted, if such a coincidence is unequivocally excluded.