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

Introduction: Primary non-Hodgkin’s lymphoma arising from the skull base (PLSB) is a rare manifestation of lymphoma, which is most commonly subclassified as diffuse large B-cell lymphoma (DLBCL). This lesion most frequently arises from the clivus, and as such, demonstrates a unique clinical presentation involving cranial nerve (CN) VI (abducens nerve) palsy, manifesting as lateral rectus paresis and horizontal diplopia. In this case report and literature review, we document the clinical presentation and management of a case of DLBCL arising from the clivus, along with a review of current literature pertaining to DLBCL of the skull base with the aim of increasing awareness and decreasing time to diagnosis of this rare pathology.

Methods: Systematic review and illustrative case example.

Results: A 71-year-old man presented with a month-long history of refractory headache, fatigue, and night sweats, with MRI brain revealing a 4.8-cm lesion of the clivus involving the adjacent petrous apex and occipital condyle ([Fig. 1]). He then developed left ophthalmoplegia (lateral rectus palsy) and personality changes prior to transsphenoidal subtotal resection and biopsy, which exhibited the pathologic and immunohistochemical characteristics of DLBCL, staining positive for CD19 and CD20, among other pathologic markers ([Fig. 2]). He underwent rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) therapy with subsequent improvement of his symptoms and continues to follow up at our institution.

Systematic review identified 18 publications meeting inclusion criteria. Pathologically confirmed DLBCL of the skull base was observed in 53 patients, with a mean age of 58 years upon presentation and slight male predominance. The most common primary location for this neoplasm was the clivus (78%), with the most frequent presenting symptoms being CN VI palsy (63%) and headache (32%). The most predominantly reported immunohistochemical markers were CD20 and CD45 positivity; treatment generally involves biopsy (often via transsphenoidal approach) followed by adjuvant chemo and/or radiotherapy. The majority of patients in this review experienced disease-free survival at the time of case publication.

Conclusion: Diffuse large B-cell lymphoma is the most common subclassification of primary non-Hodgkin's lymphoma arising from the skull base, most often originating from the clivus and resulting in a CN VI (abducens nerve) palsy. The literature regarding this neoplasm remains limited, although current publications indicate a unique clinical presentation and immunohistochemical profile. Treatment generally involves biopsy, followed by chemo and/or radiotherapy, with a favorable clinical outcome. Further study is warranted to determine the incidence of this rare neoplasm.

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Artikel online veröffentlicht:
05. Februar 2024