Idiopathic Orbital Inflammation, thyroid-related orbitopathy, sarcoidosis, ANCA-associated vasculitis, systemic amyloidosis
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
Preliminary dx: “blue cell tumor”
Final dx: Extramedullary Plasmacytoma
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.
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.
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.
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Sandy Zhang-Nunes, MD, Director of Oculofacial Plastic Surgery, Assistant Professor of Ophthalmology email@example.com