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

Sep 13, 2017
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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.”

Abstract:

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  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.