Published case study – Fibrin associated EBV-positive Diffuse Large B-Cell Lymphoma within a frontotemporal arachnoid cyst with hemorrhage

On 7th August 2017, in a Letter to the Editor of Acta Neuropathologica Communications, Daniel Kirschenbaum from the Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland, and colleagues reported a case of a male patient, 81 years of age, who presented with intermittent tremor and gait ataxia.

Key Highlights:
  • On MRI findings, a right frontotemporal arachnoid cyst with focal bleeding suspected
  • Cyst was washed out and lining was resected; whole-body PET-CT showed no other lesions
  • Due to patient’s relatively advanced age, rituximab and lenalidomide therapy was initiated
  • Hematoxylin-eosin staining identified small, discohesive islands of large atypical cells against a background of abundant fibrin
  • Mib-1 proliferation index of >80%; strongly positive for CD30, BCL2, IRF-4; BCL6 focally positive; negative for CD10, TdT, pancytokeratin, and melanocytic markers
  • In situ hybridization identified presence of non-coding Epstein-Barr Virus (EBV)-associated RNA in most of the atypical cells
  • Immunohistochemistry found C-MYC was expressed in over half of cells; no C-MYC rearrangement detected by FISH
  • Analysis using PCR found monoclonal rearrangement of IgH gene
  • Diagnosed as fibrin associated EBV-positive Diffuse Large B-Cell (DLBCL)

The authors state that this case they report is the first of a fibrin associated DLBCL in an arachnoid cyst with hemorrhage. In rare cases, arachnoid cysts may contain primary or metastatic tumors. In most cases, surgery is often curative; however, the current case was given rituximab and lenalidomide. The authors stress that “greater awareness is important in order to more accurately assess the natural history of this entity.”




  1. Kirschenbaum D. et al. Fibrin-associated diffuse large B-cell lymphoma in a hemorrhagic cranial arachnoid cyst. Acta Neuropathologica Communications. 2017 Aug 7;5(1):60. DOI: 10.1186/s40478-017-0463-3.