Angiomatoid fibrous histiocytoma of bone: A calcifying sclerosing variant mimicking osteosarcoma

David Chas Mangham, Ann Williams, Radhesh K. Lalam, Marie Anne Brundler, Michael G. Leahy, Wim Paul Cool

    Research output: Contribution to journalArticlepeer-review


    Angiomatoid fibrous histiocytoma is a neoplasm of uncertain histogenesis, which most commonly arises in the subcutaneous tissue of the extremities of children and young adults. We report the first case of a calcifying sclerosing variant of this entity. This case arose in bone-a site where there has been just 1 previously published case of typical (nonsclerosing/mineralizing) angiomatoid fibrous histiocytoma. The patient presented with the classical paraneoplastic syndrome that can occur with this tumor type and, apart from the described extracellular matrical features, displayed typical histologic features. Due to the rarity of angiomatoid fibrous histiocytoma at this site and the presence of matrical sclerosis/mineralization, this case raised the important differential diagnosis of osteosarcoma, both histologically and radiologically. The diagnosis was confirmed by molecular analysis; fluorescence in situ hybridization for EWSR1 gene disruption, and reverse transcription-polymerase chain reaction using newly designed primers on both frozen and decalcified, paraffin-embedded tissue samples showing an EWSR1-ATF1 translocation. It is important to recognize that this relatively indolent tumor can arise in bone and that it can contain focally mineralized hyalinized sclerotic matrix to avoid making the serious misdiagnosis of high-grade osteosarcoma and administering aggressive systemic chemotherapeutic treatment. Copyright © 2010 by Lippincott Williams & Wilkins.
    Original languageEnglish
    Pages (from-to)279-285
    Number of pages6
    JournalAmerican Journal of Surgical Pathology
    Issue number2
    Publication statusPublished - Feb 2010


    • Angiomatoid fibrous histiocytoma
    • ATF1
    • Bone tumor
    • EWSR1
    • Osteosarcoma


    Dive into the research topics of 'Angiomatoid fibrous histiocytoma of bone: A calcifying sclerosing variant mimicking osteosarcoma'. Together they form a unique fingerprint.

    Cite this