title: Cutaneous epithelioid sarcomalike (pseudomyogenic) hemangioendothelioma: a little-known low-grade cutaneous vascular neoplasm.
abstract: OBJECTIVE
  Epithelioid sarcomalike (pseudomyogenic) hemangioendothelioma (ES-H) is a recently described and little-known vascular neoplasm that frequently presents with dermatologic lesions. Histopathologic characterization includes sheets or fascicles of plump, spindled and epithelioid, rhabdomyoblastlike neoplastic cells involving the dermis and often extending to subcutaneous tissue. Immunohistochemical analysis reveals neoplastic cells that show a constant immunophenotype characterized by immunoreactivity for cytokeratins and endothelial markers.
  
  
  METHODS
  We described the clinical, histopathologic, and immunohistochemical features of 2 cases of cutaneous ES-H. Clinical examination revealed multifocal lesions that consisted of erythematous nodules on the leg and foot in case 1 and small perioral papules in case 2. Neoplastic cells had a rhabdomyoblastic appearance, with large nuclei and ample eosinophilic cytoplasm. Immunohistochemical analysis revealed expression of cytokeratin AE1/AE3, CD31, ERG, and FLI-1, with focal and weak positivity for CAM 5.2 and smooth muscle actin. The nuclei of neoplastic cells showed intact expression of INI-1. This immunoprofile, especially the ERG positivity, demonstrated the endothelial nature of proliferating cells.
  
  
  CONCLUSIONS
  We recommend adding the low-grade neoplasm ES-H to the large list of cutaneous vascular proliferations. Dermatologists should be aware of this low-grade cutaneous vascular tumor.
authors.affiliation: Department of Dermatology, Fundación Jiménez Díaz, Universidad Autonoma de Madrid, Madrid, Spain. lrequena@fjd.es
authors.forename: Luis
authors.initials: L
authors.lastname: Requena
authors.forename: Carlos
authors.initials: C
authors.lastname: Santonja
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Martinez-Amo
authors.forename: Carlos
authors.initials: C
authors.lastname: Saus
authors.forename: Heinz
authors.initials: H
authors.lastname: Kutzner
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000378:metabolism; D008297:Male; D012867:Skin / Q000378:metabolism / Q000473:pathology*; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D007633:Keratins