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LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: A Curious Case of Proptosis

Haghighi Zhang-Nunes
Presenter: Arezu Haghighi, MD Discussant: Sandy Zhang-Nunes, MD
 

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
Figure 1
Figure 1: Left panel shows orbital congestion, LUL swelling and limitation of infraduction, adduction, and abduction of the left eye. Right image shows worm’s eye view reveals proptosis OS.

 

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
Figure 2
Figure 2: A. CT orbits w/ contrast shows enhancing intraconal lesion, and absence of bony/lytic lesions; B. Orbitotomy reveals a firm, vascular mass; C. H&E stain of orbital biopsy demonstrates copious lymphocytes;
D. CD138 stain is highly positive.

 

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.
Figure 3
Figure 3: Additional potential manifestations of MM: A. Conjunctival infiltration; B. Uveal infiltration; C. Retinal hemorrhages due to anemia

 

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.
Figure 4
Figure 4: Worsening proptosis two days after starting radiation therapy. Patient was treated with adjuvant dexamethasone for the swelling.

 

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.
Figure 5
Figure 5: s/p surgical resection + radiotherapy (~two mos after presentation) showing marked improvement in orbitopathy OS.

 

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.

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Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel
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