Giant benign lymphangioendothelioma with positive expression of Wilms tumor 1: A case report

Abstract

Benign lymphangioendothelioma (BL, acquired progressive lymphangioma) is a rare, slow-growing lymphatic tumor, first described 40 years ago, with fewer than 50 published cases. Clinically, it presents as a skin-colored or erythematous patch. Definitive diagnosis requires histopathological examination. The immunohistochemical staining

Surgical Treatment for Benign Lymphangioendothelioma After Two Incomplete Excisions: A Case Report and Literature Review

“…Since the majority of evidence indicates that BL is a lymphatic malformation, sirolimus, which inhibits the incidence and progression of BL by targeting VEGFR-3, has been used to treat BL and has achieved satisfactory outcomes. 26 However, some cases of BL with lesions larger than 60 cm have shown positive WT-1 expression, 34 , 35 a marker of proliferation and neoplasia rather than a malformation, indicating that BL may develop a proliferative capacity in the slow enlargement process.…”

“…27,28 Another hypothesis regarding BL's pathogenesis suggests that it may be a hamartoma of intermediately differentiated lymphatic vessels, blood vessels, and smooth muscle, given that lymphatic endothelial markers, various blood endothelial markers, type IV collagen, and desmin have been found to surround the vascular channels in many BL cases. 29 In terms of the nature of BL, it is widely accepted that BL is a lymphatic vascular malformation rather than a true neoplasm, as demonstrated by the absence of WT-1 23,30,31 and D2-40 expression 18,23,[32][33][34] in endothelial cells. In the present case, positive D2-40 expression in endothelial cells further supports this view.…”