Symplastic/pseudoanaplastic giant cell tumor of the bone. Academic Article uri icon

Overview

abstract

  • OBJECTIVE: 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. MATERIALS AND METHODS: 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. RESULTS: 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 %). CONCLUSIONS: 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.

publication date

  • March 28, 2016

Research

keywords

  • Bone Neoplasms
  • Giant Cell Tumor of Bone
  • Histones

Identity

PubMed Central ID

  • PMC4996350

Scopus Document Identifier

  • 84961674008

Digital Object Identifier (DOI)

  • 10.1007/s00256-016-2373-z

PubMed ID

  • 27020452

Additional Document Info

volume

  • 45

issue

  • 7