Lymphatic differentiation in renal angiomyolipomas

Renal angiomyolipomas are mesenchymal neoplasms with varying proportions of smooth muscle, adipose tissue, and abnormal blood vessels. Although the presence of lymphangiomatous-like foci is frequently noted in large series of angiomyolipoma, lymphatic differentiation has not been previously studied. Twelve angiomyolipomas from 10 patients were identified. All tumors expressed a melanocytic marker, HMB-45 or Melan-A. Twenty-eight paraffin blocks (1-4 per tumor) were stained for lymphatic endothelial cell markers, podoplanin, and D2-40, and the presence and distribution of lymphatic differentiation were recorded. The angiomyolipomas ranged from typical triphasic tumors to leiomyoma-like and lipoma-like tumors. All 12 tumors showed positive staining with podoplanin, and all 6 tumors stained for D2-40 were also positive, indicative of lymphatic differentiation. Lymphatic differentiation was variably observed throughout the tumors. It was most prevalent in myoid areas of the triphasic angiomyolipomas and in the leiomyoma-like variant, but infrequent and widely scattered within the adipose regions of triphasic angiomyolipoma and in the lipoma-like variant. The lymphatics were usually small, often irregularly shaped, and isolated vessels in fat, whereas in myoid regions lymphatics were clustered and in some areas formed a sinusoidal or labyrinth-like pattern. Lymphatics were commonly adjacent to abnormal arteries. However, unlike the lymphatics in the normal renal cortex, a consistent adventitial association was not observed and the clustering around arteries is regarded as reflecting the myoid regions that typically exist in these areas. In conclusion, lymphatic differentiation is common in angiomyolipomas, preferentially located in myoid regions. These data expand the mesenchymal pluripotential profile of renal angiomyolipomas.