Extramedullary Plasmacytoma (Grand Rounds)

From EyeWiki
Original article contributed by: Jesse L. Berry, MD
All contributors: Jesse L. Berry, MD
Assigned editor:
Review: Assigned status Up to Date by Jesse L. Berry, MD on February 15, 2017.


Proptosis intro.JPG

History

  • 56-year-old Chinese female with multiple myeloma presented with one month of progressive left eye proptosis, rapidly worsening over past several days
  • Had completed six cycles of induction chemotherapy with CyBorD

Exam Findings

  • Proptosis, ptosis, orbital congestion OS
  • Optic neuropathy OS
  • Ocular dysmotility OS
Proptosis 1.JPG

Differential Diagnosis

  • Neoplastic
    • Lymphoproliferative (plasmacytoma vs lymphoma), optic nerve glioma/ meningioma, rhabdomyosarcoma, neurofibroma
  • Infectious
    • Orbital cellulitis, mucormycosis, aspergillosis
  • Vascular
    • Cavernous malformation, venolymphatic malformation, AVM, C-C fistula, hemangiopericytoma
  • Inflammatory
    • Idiopathic Orbital Inflammation, thyroid-related orbitopathy, sarcoidosis, ANCA-associated vasculitis, systemic amyloidosis

Additional Investigations

  • CT orbits w/ contrast showinga heterogeneous, highly vascular intraconal mass
  • Orbital biopsy – Transconjunctival orbitotomy revealed a multilobulated firm mass with numerous large vessels. Pathology consisted of sheets of plasma cells, + CD138, + kappa light chain
Proptosis 2.JPG

Diagnosis

  • Preliminary dx: “blue cell tumor”
  • Final dx: Extramedullary Plasmacytoma

Pathophysiology

  • Plasmacytoma is a plasma cell tumor arising in tissue outside of bone, and may or may not be associated systemic multiple myeloma.
  • Multiple myeloma can have orbital manifestations; however, more commonly involves bony metastases, and only rarely affects the soft tissues.
  • It is important to recognize that patients with MM can experience soft tissue metastases, and if initial CT does not show bony lesions, then an MRI should be considered to obtain greater detail.
  • The differential diagnosis is necessarily broad in such patients, and due to chronic immunosuppression, needs to include secondary malignancies and infectious causes.
Proptosis 3.JPG

Treatment

  • MM is generally treated with chemotherapy +/- autologous stem cell transplantation. So far, patient has received only CyBorD for her systemic disease.
  • Extramedullary plasmacytoma can respond well to systemic steroids and adjuvant radiation (see Figure 4).
  • Patient developed extramedullary orbital plasmacytoma despite on-going systemic chemotherapy with CyBorD. The orbital metastasis responded well initially to steroids; however, the effect was short-lived, and required subsequent orbital radiotherapy.
Proptosis 4.JPG

Prognosis and Future Directions

  • Overall prognosis for Stage III multiple myeloma is ~ 24-months survival. Patient experienced complete resolution of her orbital plasmayctoma (see Figure 5) but went on to develop plasmacytomas in the bone,soft tissue, muscle, and GI tract.
  • Her oncologist is planning a change in chemotherapy regimen with possible utilization of autologous stem cell transplantation.
Proptosis 5.JPG

References

  • Priluck JC et al. Spectral-domain optical coherence tomography of Roth spots in multiple myeloma. Eye. (2012) 26, 1588-1589.
  • Kim W et al. Hyperviscosity-Related Retinopathy in Waldenstroom’s Macroglobulinemia. New Engl J Med (2016); 374:73.
  • Tailor TD et al. Orbital Neoplasms in Adults: Clinical, Radiologic, and Pathologic Review. Radiographic’s (2013). 33: 1739-1758.
  • Gerecke C et al. The Diagnosis and Treatment of Multiple Myeloma. Dtsch Arztebl Int (2016); 113: 470-6.
  • Chin KJ et al. Ocular manifestations of multiple myeloma: Three cases and a review of the literature. Optometry (2011) 82, 224-230.
  • Sasaki R et al. Multi-Institutional Analysis of Solitary Extramedullary Plasmacytoma of the Head and Neck Treated with Curative Radiotherapy. Int. J. Radiation Oncology Biol. Phys. (2012). V. 82 (No. 2) 626-634.
  • Klintworth GK et al. Analysis of Corneal Crystalline Deposits in Multiple Myeloma. Am J Ophthalmol (1978). 86 (3): 303-313.

Contact

  • Sandy Zhang-Nunes, MD, Director of Oculofacial Plastic Surgery, Assistant Professor of Ophthalmology, zhangnun@med.usc.edu
  • Arezu Haghighi, MD, PGY-2 Ophthalmology resident, arezu.haghighi@med.usc.edu