Angiomatoid Fibrous Histiocytoma

Angiomatoid Fibrous Histiocytoma

Khin Thway, BSc, MBBS, FRCPath

Hematoxylin & eosin shows a circumscribed lesion with a pronounced lymphoid cuff, including prominent germinal centers. This appearance may mimic that of a tumor metastatic to a lymph node.

Hematoxylin & eosin shows cellular proliferations of spindle cells in loose fascicles and areas image with a storiform pattern. The cells have bland ovoid nuclei in this angiomatoid fibrous histiocytoma.



  • Angiomatoid fibrous histiocytoma (AFH)


  • Originally angiomatoid “malignant” fibrous histiocytoma

    • Term “malignant” removed due to indolent behavior

    • Unrelated to malignant fibrous histiocytoma (MFH)/pleomorphic sarcoma group of neoplasms


  • Rare neoplasm of intermediate biologic potential with 3 characteristic translocations


Lineage Unknown

  • Endothelial or histiocytic differentiation not proven

  • Desmin expression suggests myoid or myofibroblastic differentiation

  • Postulated nodal fibroblastic reticulum cell differentiation



  • Incidence

    • Rare

    • Accounts for approximately 0.3% of all soft tissue neoplasms

  • Age

    • Infancy to 8th decade

    • Predominantly in children and young adults

  • Gender

    • Slight female predilection


  • Extremities

  • Trunk

  • Head and neck

  • 1 primary intracerebral case reported

  • Usually superficial

    • Deep dermis and subcutis

  • Few arise deeply


  • Slowly growing, painless mass

    • Usually small

      • Most often 2-4 cm

  • Constitutional symptoms in subset

    • e.g., malaise, pyrexia, and anemia

    • Possible tumoral cytokine production


  • Surgical approaches

    • Wide excision

      • Usually curative

    • Radiotherapy and chemotherapy

      • For rare metastatic or unresectable tumors


  • Excellent in most cases

    • Majority of lesions indolent

  • Regional recurrence rate up to 15%

  • Metastasis rate of approximately 1%

    • Rare cause of death

  • No firm morphologic or clinical indicators of behavior

    • Infiltrative margin and deep location can predict recurrence


General Features

  • Firm

  • Circumscribed

  • Blood-filled cystic cavities

Sections to Be Submitted

  • Lesion should be thoroughly sampled

    • Features, such as lymphoid cuff, may only be present focally

  • Small lesions should be submitted in entirety


Histologic Features

  • Circumscribed

  • Lobulated

  • Fibrous pseudocapsule

    • Dense peripheral lymphoplasmacytic cuff in up to 80%

  • Cellular tumor

    • Cells with bland, vesicular, ovoid to spindled nuclei

    • Sheets

    • Short fascicles

    • Occasional storiform patterns

    • Ovoid or spindle forms may predominate

    • Mitoses infrequent

  • Hemorrhagic cavities

    • No endothelial lining

  • Some show marked pleomorphism and mitotic activity

  • Giant cells in some cases

Predominant Pattern/Injury Type

  • Circumscribed

  • Cystic, macroscopic

Predominant Cell/Compartment Type

  • Mesenchymal



  • Desmin positivity

    • Approximately half of cases

    • Strong cytoplasmic expression

    • Scattered desmin(+) cells may be present within lymphoid proliferation

    • Tumors negative for skeletal muscle markers

      • e.g., myogenin and MYOD1

  • Epithelial membrane antigen (EMA)

    • Just under half of cases

  • CD68

    • Frequent but nonspecific

  • CD99

    • Frequent but nonspecific

  • Very occasional “intermediate” CD34 expression reported

    • Usually negative

    • Other vascular endothelial markers also usually absent


Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiomatoid Fibrous Histiocytoma
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