Orbital and lacrimal gland progressive transformation of germinal centres--an underdiagnosed entity?

Br J Ophthalmol. 2012 Sep;96(9):1242-5. doi: 10.1136/bjophthalmol-2012-301574. Epub 2012 Jul 11.

Abstract

Aims: Progressive transformation of germinal centres (PTGC) is a reactive process of undetermined aetiology. Although usually seen as nodal disease, it can occur at extra-nodal sites, such as oral cavity, gastrointestinal tract and skin. To determine if PTGC occurs in the orbit, we reviewed cases of 'florid reactive lymphoid hyperplasia' affecting this region.

Methods: The cases were retrieved and were investigated using a panel of immunohistochemical stains and PCR clonality studies. Cases were jointly assessed by a specialist ophthalmic pathologist and a haematopathologist.

Results: Eight cases were retrieved from file, and all showed morphological features that fell within the spectrum of changes seen in PTGC. A diagnosis of PTGC was made in cases with expanded germinal centres showing >90% infiltration by mantle cell lymphocytes. Cases where there were other PTGC features, but <90% infiltration by mantle lymphocytes, were classified as incomplete or focal PTGC.

Conclusions: The authors observed the spectrum of changes previously described in nodal tissue, finding follicular hyperplasia, follicular lysis and PTGC in the orbit. They also identified one specimen that harboured a malignant lymphoma. The study demonstrated that lymphoid proliferations within the orbit can be difficult to evaluate. The authors recommend that such proliferations are investigated with a broad panel of immunohistochemical stains together with PCR where appropriate, and that there should be a low threshold for referral to a specialist haematopathologist.

MeSH terms

  • Adult
  • Aged
  • Biopsy
  • Cell Transformation, Neoplastic / pathology*
  • Databases, Factual
  • Female
  • Germinal Center / pathology
  • Humans
  • Lacrimal Apparatus / pathology*
  • Lymphoma / pathology*
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Lymphoma, Mantle-Cell / pathology*
  • Male
  • Middle Aged
  • Orbital Neoplasms / pathology*
  • Young Adult

Supplementary concepts

  • Progressive Transformation of Germinal Centers