Immunoglobulin D-Lambda Multiple Myeloma Initially Presenting in the Sphenoid Sinus, Orbital Apex, and Skull Base: A Systematic Review with a Case Report.

Objectives  Multiple myeloma (MM) with initial manifestations in the sphenoid sinus, orbital apex, and skull base is exceedingly rare. A systematic review was conducted to investigate the epidemiology and advancements . Methods  Relevant cases were identified by searching CNKI, WanFang Data, CQVIP databases, PubMed, Embase, and Web of Science. Additionally, we present a case of IgD-λ (immunoglobulin D-lambda) MM with initial symptoms of dizziness, unilateral pain, blindness, and ophthalmoplegia, leading to a 4-month overall survival. Strictly based on PRISMA standards, we included and summarized existing cases and reflected our case. Results  Our systematic review includes 34 case reports, revealing 67.6% of patients initially presented with diplopia and 44.1% underwent endoscopic procedures, notably with only two cases of IgD-λ subtype. In our case, we performed an endoscopic wide trans-ethmoidal sphenoidotomy and biopsy of the skull base and orbital apex lesion. Postoperative pathology confirmed a highly active plasmacytoma, clinically diagnosed as IgD-λ MM with a TP53 deletion mutation and multiple extramedullary metastases. A range of diagnostic tools was employed, including hemoglobin, immunoglobulin, urinary protein analysis, positron emission tomography-computed tomography (CT), bone marrow cytology, and gene detection. Conclusion  The subtle clinical manifestations of IgD-λ MM in the paranasal sinuses and skull base hinder early diagnosis. There is a paucity of literature describing MM initially presenting in these locations. CT/magnetic resonance scans are necessary to identify characteristic bone destruction. An endoscopic approach is popular for tissue biopsy. Bone marrow biopsy with a smear, serum or urine protein electrophoresis, and immunofixation electrophoresis are crucial upon the appearance of target organ damage.