Primary Lymphoma of the Skull Base in the Chinese: Clinical, Radiological, Pathological, and Therapeutic Experience in a Series of 8 Patients

Abstract

Primary lymphoma of the skull base (PLSB) is an extremely rare neoplasm and not much is known regarding the clinical features of, treatment strategies for, and prognoses of these lesions. We investigated the manifestations and clinical outcomes of Chinese patients with PLSB. We retrospectively reviewed the data from 8 consecutive patients with PLSB who had been treated at our center from October 2008 to December 2016 and analyzed their clinical features, treatment strategies, and prognoses. Of the 8 patients, 3 were men and 5 were women, with a male/female ratio of 0.6. The median age was 53.5 years. In most instances, the onset was characterized by headache, followed by dysfunction of the cranial nerves. The most frequently affected region was the cavernous sinus. On magnetic resonance imaging, the main characteristic was that the internal carotid artery was encased by the tumor without an obvious tendency to be deformed, dislocated, or narrowed. All patients underwent surgical biopsy via a transsphenoidal or sublabial-transmaxillary approach. The pathological diagnosis was non-Hodgkin lymphoma for all 8 patients, with diffuse large B-cell lymphoma in 7 patients. The patients underwent chemotherapy and involved-field radiotherapy. The median overall survival was 52 months, and the 1-year progression-free survival rate was 87.5%. PLSB is a rare disease and a diagnostic challenge, with most cases in the Chinese due to diffuse large B-cell lymphoma. We found that the incidence peaked in middle and old age, and the cavernous sinus was usually affected. Multidisciplinary treatment involving surgical biopsy, chemotherapy, and radiotherapy was an effective therapeutic strategy.