| デスモイド型線維腫症 desmoid-type fibromatosis |
| Differential Diagnosis |
中間悪性で粘液性・線維性成分を伴う間葉系腫瘍
・GIST spindle-myxoid, epitherial-myxoid
・Desmoid-type fibromatosis
・SFT
・Mixofibrosarcoma
・Leiomyosarcoma, myxoid type
悪性リンパ腫
診断
Desmoid-type fibromatosis of the extraperitoneal space (originated from gastrosplenic ligament suspected.)
Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery
: Radiologic- Pathologic Correlation RadioGraphics 2006; 26:245−264
・melting insinuation
・keloidal fiber formation
・thin-walled veins
・thick-walled small arteries
・perivascular microhemorrhages
| Desmoid-type fibromatosis |
：Fletcher, C. et al. WHO Classification of Tumours of Soft Tissue and Bone. Fourth Edition, 2013.
・性差なし extra andominal>>abdominal
・Mesenteric fibromatoses: 特発性またはGardner 型のFAP; familial adenomatous polyposis Intermediate(locally aggressive)
・局所破壊的: 再発率は約50％ 多くは深部軟部組織由来
・転移はない 多くは無症状 Abdominal lesion：境界明瞭
・広範な粘液状病巣がみられることあり, 5−10cm
・病理組織像 紡錘状細胞・膠原線維・様々な径の血管と血管周囲の浮腫・硝子様変化（腹部内病変で多い）
・粘液状変化が広がる
・免疫組織化学所見 MSA,SMA+,desmin- S100- β-catenin++70-75% FAP 例で多くみられる Fibroblast が豊富
特にmyofibroblastic なものが構成要素の主体
Markku Miettinen, et al. Tumors of the soft tissues 20 AFIP Atlas of tumor pathoology Series4, 2014
・Scar
・Gardner associated fibroma CD34 less cellular more collagenous
・Desmoplastic fibroblastoma(collagenous fibroma)
・Low-grade fibromyxoid sarcoma
・Low-gradefibrosarcoma
・Intra-abdominal fibromatosis
・Idiopathic retroperitoneal fibrosis
・Gastrointestinal stromal tumor GIST CD34 CD117 SAM desmin
Therapy
・Surgery
・Radiation
・Cytotoxic agents: anthracyclines
・Molecular targeted agents: imatinib, IFN and antiestrogen hormonal therapy; tamoxifen
GISTs
・GISTs within the mesentery occur as an extension of a small bowel GIST, metastatic disease, or, in rare cases, a primary mesenteric GIST
・At microscopic analysis, GISTs may be composed of spindle cells that closely resemble those of mesenteric fibromatosis, but GIST spindle cells are much more cellular and without abundant collagenous stroma
・Mesenteric fibromatosis is typically negative for cell markers CD34 and CD117, in contrast to GISTs
references
- Fletcher, C. et al. WHO Classification of Tumours of Soft Tissue and Bone. Fourth Edition, 2013.
- H DeMeo, et al. Anatomic CT demonstration of the peritoneal spaces, ligaments, and mesenteries: normal and pathologic processes. Radiographics, 15:755-769, 1995.
- Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery: Radiologic-Pathologic Correlation RadioGraphics 2006; 26:245−264
- Ellen M. et al. Solid Tumors of the Peritoneum, Omentum, and Mesentery in Children:Radiologic-Pathologic Correlation. RadioGraphics, 35:521−546, 2015.
- Verghese George et al. Chronic Fibrosing Conditions in Abdominal Imaging.RadioGraphics, 33:1053−1080, 2013.
- Atul B. et al. A to Z of Desmoid Tumors. AJR, 197:W1008-W1014, 2011.
- 青木隆敏：線維性・粘液性骨軟部腫瘍の画像診断と病理, 臨床画像 :30: 778-784,2014
- H.Fritsch, W. Kuhnel: Taschenatlas der anatomie 5th. edition, 2005
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