Giant-cell-poor giant cell tumor of bone: Report of two cases and literature review Review


Authors: Yakoub, M. A.; Torrence, D.; Hwang, S.; Bartelstein, M.; Healey, J. H.; Hameed, M.
Review Title: Giant-cell-poor giant cell tumor of bone: Report of two cases and literature review
Abstract: Giant cell tumor of bone (GCTB) is a locally aggressive tumor that shows predilection for the metaphysis/epiphysis of long bones, with an incidence of 4–5% of primary bone tumors. GCTB shows two main populations of cells: mononuclear cells and non-neoplastic multi-nucleated giant cells, with or without fibrous background. On the other hand, giant-cell-poor GCTB are rare with only few reports in the literature. These cases offer a diagnostic challenge, given the absence of giant cells and such cases have consistently been shown to harbor the H3F3A gene mutation by sequencing. The H3.3 G34W mutation-specific monoclonal antibody has shown high specificity in the diagnosis of GCTB. Two cases of giant-cell-poor GCTB are presented in this study, in which giant cells were absent or sparse and the diagnosis of GCTB was confirmed by the expression of H3.3 G34W monoclonal antibody in the mononuclear cells by immunohistochemistry. Whether this represents a histologic variant of GCTB or partial involution of GCTB is not yet fully understood; however, an immune response, infectious/inflammatory reaction, and/or anti-tumor cytokine production have been purported to be factors inciting disease regression in GCTB. © 2023, The Author(s), under exclusive licence to International Skeletal Society (ISS).
Keywords: immunohistochemistry; bone neoplasms; bone tumor; genetics; case report; diagnostic imaging; monoclonal antibody; antibodies, monoclonal; histone; histones; giant cell tumor of bone; osteoclastoma; humans; human; giant-cell-poor giant cell tumor of bone; h3f3a gene mutation; involuted giant cell tumor of bone
Journal Title: Skeletal Radiology
Volume: 52
Issue: 9
ISSN: 0364-2348
Publisher: Springer  
Date Published: 2023-09-01
Start Page: 1791
End Page: 1798
Language: English
DOI: 10.1007/s00256-023-04292-w
PUBMED: 36781420
PROVIDER: scopus
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF. Corresponding author is MSK author Meera Hameed -- Source: Scopus
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MSK Authors
  1. Meera Hameed
    281 Hameed
  2. Sinchun Hwang
    96 Hwang
  3. John H Healey
    547 Healey