Primary Diffuse Large B-Cell Lymphoma of the Clivus: Systematic Review and Illustrative Case Example

Oklahoma BACKGROUND: Primary non-Hodgkin's lymphoma arising from the skull base is a rare entity most commonly subclassified as diffuse large B-cell lymphoma (DLBCL). This lesion often arises from the clivus and demonstrates a cranial nerve (CN) VI palsy. In this case report and literature review, we document the clinical presentation and management of a case of clival DLBCL, along with a review of current literature pertaining to DLBCL of the skull base. METHODS: A retrospective chart review and systematic literature search using the PubMed and Ovid MEDLINE databases were conducted. Presenting symptomatology, neoplasm location, immunohistochemistry, and follow-up data were extracted from each work. RESULTS: A 71-year-old man presented with a month-long history of headache, fatigue, night sweats, and left lateral rectus palsy, with magnetic resonance imaging revealing a lesion of the clivus. He underwent biopsy and subtotal resection, in which histopathologic and immunohistochemical characteristics were consistent with DLBCL. He received rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone therapy with subsequent improvement of his symptoms. Systematic review identified 25 publications (58 patients) with a mean age of 65 years. The most common primary location for DLBCL was the clivus (26%) with resultant CN VI palsy (33%). Immunohistochemical markers were predominantly CD20 and CD45 positivity; treatment generally involves biopsy followed by adjuvant chemo and/or radiotherapy. CONCLUSIONS: DLBCL arising from the skull base often originates from the clivus and results in CN VI palsy. Current publications indicate a unique clinical presentation and immunohistochemical profile. Treatment generally involves biopsy, followed by chemo and/or radiotherapy.