title: Benign lymphangioendothelioma presenting as a giant flank mass.
abstract: Benign lymphangioendothelioma is a rare lesion of controversial etiology and a histopathologic mimic of Kaposi sarcoma and so-called 'well-differentiated' angiosarcoma. Its most typical clinical presentation is as a slowly expanding, erythematous patch or plaque; it rarely presents as a large mass. We report the second case of a giant benign lymphangioendothelioma, which arose as a serpiginous mass involving most of the flank of an elderly male with no prior radiation exposure and with a remote history of herpes zoster infection. A biopsy revealed numerous anastomosing vascular channels extending from the superficial dermis to the subcutis that were dilated to progressively slit-like in architecture. The endothelial cells lacked cytologic atypia, hobnailing, or significant mitotic activity, and human herpesvirus-8 expression was absent. Positivity for podoplanin (D2-40) was observed in the endothelial cells, supporting a lymphatic phenotype. Furthermore, the lesional cells lacked immunohistochemical expression of Wilms tumor 1, providing further support of a malformative - rather than neoplastic - pathogenesis.
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
authors.forename: Alicia M
authors.initials: AM
authors.lastname: Schnebelen
authors.affiliation: Arkansas Skin Cancer Center, Little Rock, AR, USA.
authors.forename: James
authors.initials: J
authors.lastname: Page
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
authors.forename: Jerad M
authors.initials: JM
authors.lastname: Gardner
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
authors.forename: Sara C
authors.initials: SC
authors.lastname: Shalin
mesh_terms: D000368:Aged; D001706:Biopsy; D003937:Diagnosis, Differential; D006394:Hemangiosarcoma / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D006562:Herpes Zoster / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008202:Lymphangioma / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D012514:Sarcoma, Kaposi / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D025721:WT1 Proteins / Q000378:metabolism; D000098853:Podoplanin
chemical_list: D008562:Membrane Glycoproteins; D025721:WT1 Proteins; C117468:PDPN protein, human; D000098853:Podoplanin; C583751:WT1 protein, human
keywords: Wilms tumor 1; Wolf's isotopic response; acquired progressive lymphangioma; benign lymphangioendothelioma; herpes zoster