Hematologic Malignancies Affecting the Skull Base: Two Unique Cases and a Literature Review

Objective: Hematologic malignancies affecting the skull base are rare and may present with nonspecific symptomatology. The objectives of this study were to characterize the manifestations and clinical outcomes of two cases of lymphoma at the cranial base where initial diagnoses were missed, and to review the pertinent literature.

Methods: Case reports and review of the literature.

Case 1: A 47 year-old female with an incoming diagnosis of granulomatosis with polyangiitis presented with worsening facial edema despite high dose steroids. Exam was notable for severe left-sided proptosis with restricted extraocular movements. Imaging demonstrated severe pansinusitis, left subperiosteal abscess, diffuse pachymeningeal enhancement and focal leptomeningeal enhancement in the frontal lobe with a subdural empyema. She underwent endoscopic biopsy and drainage of the subperiosteal and subdural collections. Pathology demonstrated extranodal NK/T cell lymphoma, nasal type. She was started on systemic chemotherapy with good clinical and radiographic response. She is currently undergoing radiation therapy with plans for future autologous stem cell transplant.

Case 2: A 50 year-old male who experienced an episode of aphasia and confusion while overseas and initially diagnosed with a cerebrovascular occlusion. On presentation to our institution, he was neurologically intact with left-sided hearing loss, however subsequently developed left facial palsy. CT demonstrated opacification of the left mastoid air cells and middle ear, hyperdensity in the left posterior and middle cranial fossae and a mass-like density along the left temporal lobe. MRI showed dural thickening and enhancement overlying left posterior fossa and bilateral middle cranial fossae. After retroperitoneal node biopsy, he was diagnosed with stage IV Burkitt lymphoma with CNS involvement. Systemic chemotherapy was initiated with complete facial nerve recovery and oncologic remission.

Conclusions: Hematologic malignancies may mimic a variety of more common skull base pathologies, thus posing a diagnostic challenge. Presentation may be nonspecific, including diplopia, nasal obstruction or facial nerve weakness. The literature shows that the anterolateral skull base is most commonly involved, and poor survival is associated with central nervous system involvement. Prompt identification of these cases is dependent upon appropriate clinical suspicion, which is necessary to ensure proper treatment with the assistance of a multidisciplinary oncologic team.

No conflict of interest has been declared by the author(s).