title: Malignant hepatic epithelioid hemangioendothelioma with rapid progression and fatal outcome.
abstract: A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.
authors.affiliation: The First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan. koma@suwa.jrc.or.jp
authors.forename: Yoshimichi
authors.initials: Y
authors.lastname: Komatsu
authors.forename: Tomonobu
authors.initials: T
authors.lastname: Koizumi
authors.forename: Masanori
authors.initials: M
authors.lastname: Yasuo
authors.forename: Kazuhisa
authors.initials: K
authors.lastname: Urushihata
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hanaoka
authors.forename: Keishi
authors.initials: K
authors.lastname: Kubo
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kawakami
authors.forename: Takayuki
authors.initials: T
authors.lastname: Honda
authors.forename: Keisaku
authors.initials: K
authors.lastname: Fujimoto
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Hachiya
mesh_terms: D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis / Q000556:secondary; D008875:Middle Aged; D013997:Time Factors