Soft tissue
Fibrohistiocytic tumors
Fibrohistiocytic tumors - general

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 14 September 2016, last major update August 2012

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PubMed Search: Fibrohistiocytic tumors

Definition / General
  • Tumors composed of cells with morphologic features of fibroblasts and histiocytes
  • Cells are actually fibroblasts, myofibroblasts or primitive mesenchymal cells
  • Cells are NOT histiocytes, but may exhibit phagocytic properties, including phagocytosis of neutrophils
  • Tumor cells may express histiocytic markers CD68, alpha-1-antitrypsin or alpha-1-antichymotrypsin
Classification
WHO Classification (2006) of fibrous, fibrohistiocytic and histiocytic tumours occurring in skin (superficial) and ICD-O codes:
  • Dermatomyofibroma: 8824 / 0
  • Infantile myofibromatosis: 8824 / 1
  • Sclerotic fibroma: 8823 / 0
  • Pleomorphic fibroma: 8832 / 0
  • Giant cell fibroblastoma: 8834 / 1
  • Dermatofibrosarcoma protuberans: 8832 / 3
  • Dermatofibroma (fibrous histiocytoma): 8832 / 0

WHO classification (2002) of fibrohistiocytic tumors of soft tissue (deep) and ICD-0 codes:
  • Benign
    • Giant cell tumour of tendon sheath: 9252 / 0
    • Diffuse-type giant cell tumour: 9251 / 0
    • Deep benign fibrous histiocytoma: 8830 / 0

  • Intermediate (rarely metastasizing)
    • Plexiform fibrohistiocytic tumour: 8835 / 1
    • Giant cell tumour of soft tissues: 9251 / 1

  • Malignant
    • Pleomorphic ‘MFH’ / Undifferentiated pleomorphic sarcoma: 8830 / 3
    • Giant cell ‘MFH’ / Undifferentiated pleomorphic sarcoma with giant cells: 8830 / 3
    • Inflammatory ‘MFH’ / Undifferentiated pleomorphic sarcoma with prominent inflammation: 8830 / 3