An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs

Abstract

We describe the case of a 61-year-old man presenting with subacute encephalopathy. The clinical manifestations included progressive dementia and pyramidal and extrapyramidal tract signs. Brain CT scan and MRI showed diffuse bilateral white matter changes in the cerebral hemispheres, basal ganglia, thalamus and brainstem. No contrast-enhanced lesion was observed. Peripheral blood studies, CSF analysis, and brain and muscle biopsies were nonspecific and failed to reveal diagnostic evidence of any specific disease. The patient was diagnosed with and treated for a cerebral demyelinating disorder. Post mortem examination showed diffuse infiltration of lymphoma cells without mass lesions in the extensive cerebral white and gray matter with minimal intravascular patterns, particularly in the perivascular and periventricular spaces. These findings were consistent with lymphomatosis cerebri (LC). In other visceral organs such as the lungs, liver, kidneys and adrenal glands, blood vessels were plugged by numerous neoplastic cells which were morphologically and immunohistochemically similar to those observed in the CNS, consistent with intravascular malignant lymphoma (IVL). To our knowledge, this is the first autopsy report showing the coexistence of LC and IVL. This case suggests a possible link between LC and IVL.

Lymphomatosis cerebri: diagnostic challenges and review of the literature

“…PCNSL often occurs in the setting of immunosuppression, such as organ transplantation or HIV/AIDS, the latter associated with Epstein-Barr virus infection 10. However, most reported cases of LC have occurred in immunocompetent individuals 11 12…”

Interleukin 10 Level in the Cerebrospinal Fluid as a Possible Biomarker for Lymphomatosis Cerebri

“…The final diagnosis was made following immunostaining for GFAP and other leukocyte markers. CD20 immunostaining allowed us to visualize the extent of infiltration of the lymphoma cells throughout the white matter (11,12). Hans et al subclassified diffuse large B-cell lymphoma into germinal center B-cell-like (GCB) and non-GCB profiles with immunostaining for CD10, bcl-6 and MUM1.…”

Early Diagnosis of Hepatic Intravascular Lymphoma: A Case Report and Literature Review

“…The patient was successfully treated with chemotherapy following the histological diagnosis of hepatic IVL (13). However, due to the difficulty in confirming the suspicion of liver invasion using imaging studies and biopsy methods, to date the number of cases involving the liver diagnosed in the antemortem period is lower than that observed in the postmortem period (12)(13)(14)(15)(16)(17)(18)(19)(20)(21) (Table). Our literature review of hepatic IVL cases diagnosed using imaging studies of the liver revealed that six of 10 cases were diagnosed at autopsy, and no specific findings were observed in the conventional CT studies (Table).…”