Rapid deterioration of intravascular large B-cell lymphoma with mass formation in the trigeminal nerve and multiple organ infiltration: An autopsy case report

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 62; no. 1; pp. 41 - 45
• Main Authors: Tanaka, Yuka, Momose, Shuji, Takayanagi, Natsuko, Tabayashi, Takayuki, Tokuhira, Michihide, Tamaru, Jun-ichi, Kizaki, Masahiro
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society for Lymphoreticular Tissue Research 01.01.2022; JSLRT
• Subjects: Autopsy; biopsy; bone marrow biopsy; Case Report; Fluorodeoxyglucose F18; Humans; Intravascular large B-cell lymphoma; Lymphoma, Large B-Cell, Diffuse - diagnosis; Male; Positron Emission Tomography Computed Tomography; random skin biopsy; trigeminal nerve; Trigeminal Nerve - pathology; biopsy; trigeminal nerve; random skin biopsy; bone marrow biopsy; Intravascular large B-cell lymphoma
• ISSN: 1346-4280; 1880-9952
• DOI: 10.3960/jslrt.21013

Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma characterized by the selective growth of lymphoma cells within the lumen of vessels. We describe the case of a 69-year-old male who presented with marked pain in the left facial region. Gadolinium-enhanced magnetic resonance imaging revealed a swollen left trigeminal nerve (TN) and positron emission tomography/computed tomography demonstrated fluorodeoxyglucose-only uptake at the same site. The patient had high serum lactate dehydrogenase and soluble interleukin-2 receptor levels. As random skin biopsy and bone marrow biopsy detected no abnormal pathogenesis, open biopsy of the TN was performed, revealing diffuse large B-cell lymphoma (DLBCL). However, ground glass opacities rapidly developed in both lung fields with severe respiratory failure. The patient died of progressive disease before the initiation of chemotherapy. Postmortem examination revealed widespread lymphoma cells in the lumen of vessels in multiple organs, including the lungs, excluding the bone marrow and skin. Lymphoma cells formed a mass in the TN and left lumbar plexus. A diagnosis of IVLBCL was made based on the postmortem pathological analysis. DLBCL of abnormal sites, such as the peripheral nervous system, should be considered in cases of IVLBCL as a differential diagnosis.