title: Epithelioid hemangioendothelioma presenting with severe myelofibrosis and a high serum hyaluronan level.
abstract: Epithelioid hemangioendothelioma (EHE) is a rare tumor originating from the vascular endothelium; it has an intermediate malignant potential. EHEs affect all age groups and mostly originate from the soft tissues of the extremities, lungs, and liver. Spinal EHEs, especially those occurring in the bone marrow region, are extremely rare. We report a case of EHE with massive involvement of the liver, vertebrae, and cranial bones that caused severe myelofibrosis (MF) in a 67-yr-old-male patient. Hyaluronan deposits were diffusely observed in the tumor tissue biopsies obtained from both the liver and bone marrow. Furthermore, the serum hyaluronan level increased markedly along with rapid progression of the disease. To the best of our knowledge, this is the first report of MF occurring in an EHE; hyaluronan may have played an important role in the pathogenesis of fibrosis in this case.
authors.affiliation: Department of Advanced Molecular and Cell Therapy, Kyushu University, Higashi-ku, Fukuoka, Japan. suehiro@sentan.med.kyushu-u.ac.jp
authors.forename: Youko
authors.initials: Y
authors.lastname: Suehiro
authors.forename: Yoshimichi
authors.initials: Y
authors.lastname: Tachikawa
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Abe
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
authors.forename: Koichiro
authors.initials: K
authors.lastname: Muta
authors.forename: Kenzaburo
authors.initials: K
authors.lastname: Tani
mesh_terms: D000368:Aged; D001706:Biopsy; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D019046:Bone Marrow Neoplasms / Q000097:blood* / Q000150:complications / Q000473:pathology; D018323:Hemangioendothelioma, Epithelioid / Q000097:blood* / Q000150:complications / Q000473:pathology; D006801:Humans; D006820:Hyaluronic Acid / Q000097:blood*; D008113:Liver Neoplasms / Q000097:blood* / Q000150:complications / Q000473:pathology; D008297:Male; D018204:Neoplasms, Connective and Soft Tissue / Q000097:blood / Q000150:complications / Q000473:pathology; D055728:Primary Myelofibrosis / Q000097:blood* / Q000150:complications / Q000473:pathology; D012888:Skull Neoplasms / Q000097:blood* / Q000150:complications / Q000473:pathology; D013125:Spinal Neoplasms / Q000097:blood* / Q000150:complications / Q000473:pathology
chemical_list: D006820:Hyaluronic Acid