title: Multifocal epithelioid hemangioendothelioma: case report of a clinical chamaeleon.
abstract: Epithelioid hemangioendothelioma is an extremely rare vascular bone tumor with a slow growth and poor prognosis. The term was designed to describe neoplasms that had an appearance in between hemangiomas and sarcomas. Various synonyms for epithelioid hemangioendothelioma are used clinically: low grade anaplastic angiosarcoma, cellular hemangioma, histiocytoid hemangioma and angioendothelioma. However, it represents 1% of all vascular neoplasms and is locally aggressive. We report the course of disease of a 47-year-old man who presented to our clinic with unspecific abdominal and back pain. Radiological findings revealed multiple lesions in the spine as well as liver and spleen involvement. Tumor histology of the bone and liver biopsies confirmed the diagnosis of epithelioid hemangioendothelioma. Although treatment was initiated with thalidomide, the patient developed multiple organ dysfunction syndrome (MODS) and succumbed to his disease. This case report may contribute to the data on clinical findings and natural history of this rare tumor.
authors.affiliation: Department of Radiation Therapy and Radiation Oncology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany. eboelke@aol.com
authors.forename: Edwin
authors.initials: E
authors.lastname: Bölke
authors.forename: S
authors.initials: S
authors.lastname: Gripp
authors.forename: M
authors.initials: M
authors.lastname: Peiper
authors.forename: W
authors.initials: W
authors.lastname: Budach
authors.forename: A
authors.initials: A
authors.lastname: Schwarz
authors.forename: K
authors.initials: K
authors.lastname: Orth
authors.forename: P
authors.initials: P
authors.lastname: Reinecke
authors.forename: J A P
authors.initials: JA
authors.lastname: van de Nes
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D018323:Hemangioendothelioma, Epithelioid / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013125:Spinal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D013160:Splenic Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D013577:Syndrome; D013792:Thalidomide / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
chemical_list: D020533:Angiogenesis Inhibitors; D013792:Thalidomide