Epithelioid hemangioendothelioma presenting with severe myelofibrosis and a high serum hyaluronan level
| Autor: | Yoshimichi Tachikawa, Kenzaburo Tani, Yasunobu Abe, Youko Suehiro, Koichiro Muta, Koichi Ohshima |
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| Rok vydání: | 2007 |
| Předmět: |
Male
Pathology medicine.medical_specialty Biopsy Skull Neoplasms Pathogenesis Age groups Bone Marrow Fibrosis medicine Humans Hyaluronic Acid Myelofibrosis Epithelioid hemangioendothelioma Aged Spinal Neoplasms business.industry Liver Neoplasms Soft tissue Hematology General Medicine medicine.disease Vascular endothelium medicine.anatomical_structure Primary Myelofibrosis Hemangioendothelioma Epithelioid Bone marrow Bone Marrow Neoplasms business Neoplasms Connective and Soft Tissue |
| Zdroj: | European Journal of Haematology. 79:349-353 |
| ISSN: | 1600-0609 0902-4441 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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