Skull-Base Plasmacytomas: A Systematic Review on Therapeutic Trends

Background: Skull base plasmacytomas are rare plasma cell neoplasms arising from the clivus, sphenoid bone, and petrous apex, presenting with cranial neuropathies and bearing a high risk of progression to multiple myeloma. Optimal management remains poorly defined due to their rarity. Objective: The objectives of this study are to comprehensively characterize the clinical presentation, management strategies, and outcomes of skull base plasmacytomas and identify evolving trends in the literature. Methods: A systematic search of PubMed, EMBASE, Web of Science, and Cochrane databases was conducted, yielding 92 studies (18 case series and 74 case reports) encompassing 118 patients. Data on the demographics, symptoms, imaging, histopathology, treatment, and outcomes were extracted and analyzed. Results: The median patient age was 56 years; visual disturbances (66.1%) and headache (54.2%) were the most common presenting symptoms. The middle and posterior cranial fossae were most frequently involved, and cranial nerve VI was most affected (65.7%). Surgical resection was performed in 63.6% of cases with a gross total resection in 36%. At a median follow-up of 12 months (calculated across the entire cohort of 118 patients, including the 29 who presented with a prior diagnosis of multiple myeloma), 81.4% were alive, with symptom resolution at 38.1% or improvement at 26.2%. Recurrence occurred in 19.4% of patients, and multiple myeloma developed in 25.8% of patients without a prior diagnosis. Multiple myeloma status was the only independent predictor of survival on multivariate analysis (OR = 10.14, 95% CI: 1.63–63.04, p = 0.013). Conclusions: Skull base plasmacytomas show consistent clinical patterns but variable management strategies. Surgery is increasingly utilized; yet, its survival benefit remains unclear. Prospective multicenter studies with standardized outcome reporting and molecular profiling are needed to optimize individualized care.