Clinical utility of positron emission tomography leading to rapid and accurate diagnosis of intravascular large B-cell lymphoma presenting with the central nervous system symptoms alone: A case report and review of the literature

Ryo Inagaki; Akihiro Inoue; Yukihiro Miyazaki; Kota Kanehisa; Joji Kunihiro; Takuya Kondo; Eiji Katayama; Mashio Taniwaki; Seiji Shigekawa; Hideaki Watanabe; Riko Kitazawa; Takeharu Kunieda

doi:10.25259/SNI_1175_2022

Clinical utility of positron emission tomography leading to rapid and accurate diagnosis of intravascular large B-cell lymphoma presenting with the central nervous system symptoms alone: A case report and review of the literature

Surg Neurol Int. 2023 Mar 17:14:89. doi: 10.25259/SNI_1175_2022. eCollection 2023.

Affiliations

¹ Department of Neurosurgery, Ehime University School of Medicine, Toon, Japan.
² Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University School of Medicine, Toon, Japan.
³ Division of Diagnostic Pathology, Ehime University Hospital, Toon, Japan.

Abstract

Background: Intravascular large B-cell lymphoma (IVLBCL) is a rare entity among large B-cell non-Hodgkin lymphomas and is often difficult to diagnose. We report the case of a patient with IVLBCL who presented with central nervous system (CNS) symptoms alone, in which positron emission tomography (PET) enabled a rapid and accurate diagnosis.

Case description: An 81-year-old woman was admitted to our hospital with a 3-month history of gradually progressive dementia and declining spontaneity. Magnetic resonance imaging revealed multiple hyperintense lesions bilaterally on diffusion-weighted imaging without enhancement on gadolinium-enhanced T1-weighted imaging. Laboratory findings showed elevated serum lactate dehydrogenase (626 U/L) and soluble interleukin-2 receptor (sIL-2R) (4692 U/mL). Cerebrospinal fluid (CSF) analysis showed slightly elevated levels of protein (166 mg/dL) and lymphocytic cells (29/μL), and β2-microglobulin (β2-MG) (4.6 mg/L) was highly elevated. Whole-body computed tomography revealed faint ground-glass opacities in the upper and middle lung fields and diffuse enlargement of both kidneys without lymph node swelling. ¹⁸F-fluorodeoxyglucose (FDG)-PET showed diffuse and remarkably high FDG uptake in both upper lungs and kidneys without uptake by lymph nodes, suggesting a malignant hematological disease. IVLBCL was confirmed histologically by incisional random skin biopsy from the abdomen. Chemotherapy using R-CHOP regimen in combination with intrathecal methotrexate injection was started on day 5 after admission and follow-up neuroimaging showed no signs of recurrence.

Conclusion: IVLBCL presenting with CNS symptoms alone is rare and often has a poor prognosis associated with delayed diagnosis, and various evaluations (including systemic analysis) are therefore necessary for early diagnosis. FDG-PET, in addition to identification of clinical symptoms and evaluation of serum sIL-2R and CSF β2-MG, enables rapid therapeutic intervention in IVLBCL presenting with CNS symptoms.

Keywords: 18F-fluorodeoxyglucose; Intravascular large B-cell lymphoma; Positron emission tomography; R-CHOP; Random skin biopsy.

Publication types

Case Reports