Angiomatoid Fibrous Histiocytoma

A variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is characterized by nodular subcutaneous growth and may recur after surgical excision. Characteristic histologic features include lobulated sheets of HISTIOCYTE - like cells associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a LYMPH NODE METASTASIS. May be associated with somatic fusions of the FUS, ATF1, EWSR1, and CREB1 genes. OMIM: 612160

Also Known As:

Histiocytoma, Angiomatoid Fibrous

Neoplasms: 1361496
- Neoplasms by Histologic Type: 2
  - Connective and Soft Tissue Neoplasms: 1
    - Sarcoma: 23591
      - Malignant Fibrous Histiocytoma: 754
        Angiomatoid Fibrous Histiocytoma: 24
    - Connective Tissue Neoplasms: 9
      - Fibrous Tissue Neoplasms
        Histiocytoma: 113
        Malignant Fibrous Histiocytoma: 754
        Angiomatoid Fibrous Histiocytoma: 24

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Disease

Drug/Important Bio-Agent (IBA)