Primary Cranio-Orbital Presentation of Multiple Myeloma.

Abstract

A 49-year-old man presented with left upper eyelid ptosis noted after minor head trauma. Clinical examination revealed a soft, mildly tender mass of the superior orbit with left hypoglobus and relative proptosis. He had moderate limitation in supraduction, without visual or pupillary deficit. MRI imaging showed a superior extraconal cystic mass (Fig. 1C, D), and CT demonstrated erosion of the superior orbital roof and invasion of the frontal sinus (Fig. 1A, B).FIG. 1Excisional biopsy was performed via a bifrontal craniotomy with a frontal bone flap, exposing a well-encapsulated vascular lesion eroding through the orbital roof and frontal sinus (Fig. 2A). The mass was dissected from the surrounding dura, sinus mucosa, and periorbita, and removed in toto (Fig. 2B). The frontal sinus was obliterated and a split-thickness calvarial bone graft was used to reconstruct the orbital rim and roof, secured with mini plate fixation (Fig. 2C).FIG. 2Pathological analysis revealed plasma cell neoplasm (Fig. 2D) with clonal kappa-light proliferation (Fig. 2E), consistent with an extramedullary plasmacytoma. The full-body PET scan revealed multiple lytic lesions of the clavicle, rib, and vertebrae, conferring a diagnosis of multiple myeloma. He underwent an autologous stem cell transplant and started maintenance Bortezomib therapy with a good serologic response. Multiple myeloma accounts for 10% of hematologic malignancies and presents as a malignant plasma cell proliferation leading to bone marrow destruction. There are few reports of multiple myeloma initially presenting in the orbit. Solitary plasmacytoma without systemic disease may be treated with radiation therapy. Systemic disease mandates oncologic evaluation and consideration of chemotherapy or transplant.