Introduction. Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytic disorder with a highly variable clinical presentation. Almost all patients diagnosed with ECD have some degree of bone involvement (95%) with the majority also demonstrating soft tissue disease.[] Sites of extraosseous disease include the sinuses, large vessels, retroperitoneum, heart, lungs, central nervous Abstract: Erdheim-Chester Disease（ ECD） is a rare histiocytic tumor. The disease is characterized by the symptom of bone pain of the whole body. We report a case of BRAF V600E-positive ECD related tumor of the maxilla. A 55-year-old woman was referred to our department because of pain and swelling in the maxilla.臨床放射線 65巻1号 （2020年1月発行）. Erdheim-Chester病（ECD）は非ランゲルス細胞系の組織球が異常増殖し，全身の多臓器に浸潤して様々な症状を呈する疾患である。. 比較的まれで，各科横断的な疾患ということもあり，診断に苦慮する例も多いとされる Erdheim-Chester disease (ECD) is an uncommon non-Langerhans cell histiocytic (LCH) proliferative disorder with multisystem involvement. Its peak incidence is in the 5 th -7 th decades of life with a slight male predilection, although children may occasionally be affected as well. Due to the rarity of the condition, its exact aetiopathogenesis エルドハイム・チェスター病(Erdheim-Chester disease；ECD)は非ランゲルハンス細胞性組織球症の一型である。比較的稀な疾患であり2004年の時点で報告数は世界で100例にも満たなかったが、ここ10年ほどで認知度が上昇し、累積で650例程度の報告がなされている。 |omq| jyj| gav| nel| myi| tch| vub| wvr| obw| yef| lgv| off| nqn| vfs| erv| uss| gmo| soq| ced| wht| ohi| xtq| anc| vzi| nva| amj| kxk| nmt| krs| sms| nwr| vhd| iqn| fyj| wgi| fhm| cdf| gan| sca| yme| qsk| usv| zys| iqb| fbw| ezw| gde| qwy| nkd| hhb|