Symplastic/pseudoanaplastic giant cell tumor of the bone Journal Article


Authors: Sarungbam, J.; Agaram, N.; Hwang, S.; Lu, C.; Wang, L.; Healey, J.; Hameed, M.
Article Title: Symplastic/pseudoanaplastic giant cell tumor of the bone
Abstract: Giant cell tumor of bone (GCTB) is a locally aggressive primary bone tumor. Its malignant counterpart is quite rare. Rarely, a conventional GCTB shows marked nuclear atypia, referred to as symplastic/pseudoanaplastic change, which can mimic sarcomatous transformation. Recently, somatic driver mutations of histone H3.3 exclusively in H3F3A have been described in GCTB. We report a series of 9 cases of GCTB with symplastic/pseudoanaplastic change, along with analysis of H3F3A variants. Nine cases of GCTB with symplastic change were identified. Clinico-radiological features, morphological features, and immunohistochemical stain for Ki-67 stain were reviewed. H3F3A variants were also analyzed using Sanger sequencing. Histologically, conventional giant cell tumor areas with scattered foci of markedly atypical cells were seen in all of the cases and all showed rare if any Ki-67 labeling. One patient had received denosumab treatment and another radiation therapy. Radiological features were characteristic of conventional GCTB. Mutation in H3F3A (p.Gly34Trp [G34W]) was found in 6 of the 7 cases. Clinical follow-up ranged from 6 to 208 months. Local recurrences were seen in 4 cases (44 %). GCTB with symplastic/pseudoanaplastic change is an uncommon variant of conventional GCTB, which can mimic primary sarcoma or sarcomatous transformation. These tumors possess the same missense mutation in histone H3.3 as conventional GCTB.
Keywords: mutation; sarcoma; schwannoma; lesion; giant cell tumor of bone; chondroblastoma; osteoblastoma; atypia; symplastic/pseudoanaplastic; h3f3a
Journal Title: Skeletal Radiology
Volume: 45
Issue: 7
ISSN: 0364-2348
Publisher: Springer  
Date Published: 2016-07-01
Start Page: 929
End Page: 935
Language: English
ACCESSION: WOS:000376510000007
DOI: 10.1007/s00256-016-2373-z
PROVIDER: wos
PUBMED: 27020452
PMCID: PMC4996350
Notes: Article -- Source: Wos
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MSK Authors
  1. Meera Hameed
    281 Hameed
  2. Narasimhan P Agaram
    190 Agaram
  3. Sinchun Hwang
    96 Hwang
  4. Lu Wang
    147 Wang
  5. John H Healey
    547 Healey