Dilated lymphatic vessels in tufted angioma: a potential source of diagnostic confusion

Tufted angioma (TA), an uncommon benign vascular tumor, has a variable clinical presentation, and histopathologic findings are a key component of diagnosis. The presence of lymphatic vessels suggestive of lymphangioma can occasionally be the predominant finding and cause diagnostic confusion. Nine biopsies from 7 cases of TA were studied to assess the frequency and distribution of lymphangioma-like areas. Specimens were also stained with D2-40, VEGF-A, GLUT-1, and HHV-8. In one biopsy, lymphangioma-like vessels were the main finding. In all other cases of TA, lymphatics were present in the stroma but were often overshadowed by tufts of capillaries. D2-40 highlighted the stromal lymphatics and partially stained the capillaries within tufts. VEGF-A showed diffuse nonspecific staining of epidermis and endothelial cells in all specimens. GLUT1 and HHV-8 staining were uniformly negative in all 9 specimens. Accurate diagnosis of TA has important clinical implications given its occasional association with Kasabach-Merritt phenomenon, and the presence of lymphangioma-like vessels in biopsies of vascular lesions is entirely compatible with TA.