Synchronous polyostotic solid variant of aneurysmal bone cyst involving thoracic vertebrae and harboring a novel AHNAK::USP6 gene fusion. A case report and review

Virchows Arch. 2022 Nov;481(5):805-809. doi: 10.1007/s00428-022-03362-z. Epub 2022 Jun 18.

Abstract

Aneurysmal bone cyst (ABC) is a benign bone neoplasm that usually affects the metaphysis of long bones and the posterior elements of vertebral bodies. The rearrangement of USP6 gene is present in most of primary ABC cases. Synchronous polyostotic presentation is extremely rare. All of the eight reported cases in literature have a classic ABC histomorphology, including dilated-blood filled cystic spaces separated by fibrous septa and composed of variably cellular bland fibroblasts with scattered osteoclast-like giant cells and reactive new bone formation. Herein, we report a case of a 29-year-old female with a synchronous polyostotic solid variant of ABC involving her T7-T11 posterior elements of her thoracic vertebrae with a novel AHNAK::USP6 fusion, detected by next-generation sequencing (NGS). This case is distinguished by its synchronous polyostotic presentation, solid rather than classic ABC morphology and novel AHNAK::USP6 fusion, which has not been previously reported in ABC or in any mesenchymal bone tumor.

Keywords: AHNAK; Aneurysmal bone cyst; Solid variant; USP6; Vertebrae.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bone Cysts, Aneurysmal* / genetics
  • Bone Cysts, Aneurysmal* / pathology
  • Bone Neoplasms*
  • Female
  • Gene Fusion
  • Humans
  • In Situ Hybridization, Fluorescence
  • Membrane Proteins / genetics
  • Neoplasm Proteins / genetics
  • Proto-Oncogene Proteins / genetics
  • Thoracic Vertebrae / pathology
  • Ubiquitin Thiolesterase / genetics

Substances

  • Ubiquitin Thiolesterase
  • Proto-Oncogene Proteins
  • USP6 protein, human
  • AHNAK protein, human
  • Membrane Proteins
  • Neoplasm Proteins