Primary CNS Lymphoma Presenting as Enhancing Cortical Ribboning and Hemorrhage

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Primary CNS Lymphoma Presenting as Enhancing Cortical Ribboning and Hemorrhage Lessons from Atypical Presentation of a Usual Suspect Humberto Morales1

· Nicholas Marko2 · Kritstina Brannock3 · Alisa Kanfi1 · Rekha Chaudhary4

Received: 5 July 2019 / Accepted: 17 September 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019

Introduction

Case Report

Enhancing cortical ribboning on computed tomography (CT) or magnetic resonance imaging (MRI) is rarely, if ever, considered a presenting feature of central nervous system (CNS) lymphoma. Other lesions but not lymphoma are usually included in the differential diagnosis. Hemorrhagic changes are also atypical in an untreated lymphoma and can further complicate the assessment. To our knowledge, only one prior case with similar imaging features has been reported [1]. Here, a second case is reported and the important implications of an unsuspected clinical, radiologic and pathologic correlation are discussed.

A 54-year-old male presented to the emergency room (ER) with acute onset of aphasia. Non-contrast computed tomography (CT) and CT angiography (CTA) of the head were performed to assess for an acute stroke. An enhancing left frontal lobe lesion was found (Fig. 1), which prompted a subsequent MRI (Fig. 2). Further clinical work-up revealed recent changes in personality and behavior, no history of immunosuppression and no evidence of primary malignancy. The morphology and imaging characteristics of the lesion were atypical and given the need for diagnosis and the extensive symptomatic frontal edema, the decision was made to take the patient to the operating room for biopsy with the possibility of resection. Two intraoperative smears were uncertain for a neoplastic (lymphoid) versus inflammatory process. Thus, the lesion was resected. Computed tomography (CT) revealed prominent cortical enhancement in the left frontal lobe with associated moderate vasogenic edema (Fig. 1). Magnetic resonance imaging demonstrated an enhancing cortical ribbon-like lesion in the left superior frontal gyrus (Fig. 2b) with gyral-like hemorrhagic changes (Fig. 2a and 2d), as well as moderate vasogenic edema. There was prominent cortical restricted diffusion (Fig. 2c). Mild thickening and enhancement of the adjacent falx was also noted (Fig. 2b). Differential diagnosis at the time of presentation included a high-grade glioma, atypical meningioma or an infectious/inflammatory process. Given the suspected hemorrhagic changes, a lymphoma was considered unlikely. Based on the final pathological findings (Fig. 3) a highgrade B cell lymphoma was diagnosed. The lymphoma involved the cortical and subcortical brain parenchyma (Fig. 3a). Due to the concern for an atypical meningioma, the lesion was resected en block with the adjacent falx. On pathology, the falx was also involved by lymphoma cells.

Previously presented at the 57th Annual Meeting of the American Society of Neuroradiology (ASNR), Boston, 2019.  Humberto Morales

[email protected] Nicholas Marko markonf@u