Orbital Plasmacytoma, An Uncommon Presentation of Advanced Multiple Myeloma

  • Andreia Margarida de Matos Internal Medicine Department, Coimbra Hospital and University Center, Coimbra, Portugal
  • André Goulard Internal Medicine Department, Hospital of the Divine Holy Spirit, Angra do Heroismo, Portugal
  • André Ribeiro Hematology Department, Coimbra Hospital and University Center, Coimbra, Portugal
  • Ricardo Freitas Internal Medicine and Intensive Care Unit, Coimbra Hospital and University Center, Portugal
  • Catarina Monteiro Internal Medicine and Intensive Care Unit, Coimbra Hospital and University Center, Portugal
  • Paulo Martins Intensive Care Unit, Coimbra Hospital and University Center and Faculty of Medicine of the University of Coimbra, Coimbra, Portugal

Keywords

Multiple myeloma, orbital tumour, plasmacytoma

Abstract

Introduction: Extramedullary plasmacytomas are present in 13% of multiple myeloma (MM) patients. Less than 5% of MM cases are non-secretory. The orbital location is uncommon and a minority of orbital tumours are plasmacytomas.
Description: The patient was a 71-year-old man, with right proptosis, retro-ocular pain and epistaxis with visual acuity 2/10, limitation of upper eye movement and scattered ecchymosis. Blood tests revealed severe anaemia, coagulopathy, increased serum creatinine, LDH and C-RP without improvement after antimicrobial treatment. Peripheral immunophenotyping showed 9.4% of plasma cells with intracytoplasmic clonal κ chains. IgG and λ chains were decreased with normal plasma and urine immunofixation. Orbital CT: retro-orbital superomedial tumour with bone destruction. Histology of the tumour and bone biopsy was consistent with plasmacytoma. The patient was deceased in 2 weeks.
Discussion: MM accounts for 10% of haematopoietic tumours; 7% of cases present with plasmacytomas at diagnosis. Orbital locations are rarely reported (frequently in the temporal region). Proptosis, ptosis and reduced visual acuity are common symptoms. However, orbital pain is less frequent. Most cases of MM demonstrate hypergammaglobulinaemia. Only 5% of MM cases are non-secretory.
The uncommon location, topography, symptom peculiarities and absence of monoclonality led to the diagnostic challenge of this fatal case of MM.

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References

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  • Published: 2020-01-31

    Issue: Vol 7 No 3 (view)

    Section: Articles

    How to cite:
    de Matos, A., Goulard, A., Ribeiro, A., Freitas, R., Monteiro, C., & Martins, P. (2020). Orbital Plasmacytoma, An Uncommon Presentation of Advanced Multiple Myeloma. European Journal of Case Reports in Internal Medicine, 7(3). https://doi.org/https://doi.org/10.12890/2020_001149