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.
| Original language | English |
|---|---|
| Pages (from-to) | 612-619 |
| Number of pages | 8 |
| Journal | Neuropathology |
| Volume | 31 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Dec 2011 |
| Externally published | Yes |
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Keywords
- Intravascular malignant lymphoma
- Large b-cell lymphoma
- Lymphomatosis cerebri
- Primary central nervous system lymphoma
- Progressive dementia
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Clinical Neurology
Cite this
An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs. / Hishikawa, Nozomi; Niwa, Hisayoshi; Hara, Takashi; Hara, Kazuhiro; Ito, Makoto; Shimada, Satoko; Yoshida, Mari; Hashizume, Yoshio; Murakami, Nobuyuki.
In: Neuropathology, Vol. 31, No. 6, 12.2011, p. 612-619.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs
AU - Hishikawa, Nozomi
AU - Niwa, Hisayoshi
AU - Hara, Takashi
AU - Hara, Kazuhiro
AU - Ito, Makoto
AU - Shimada, Satoko
AU - Yoshida, Mari
AU - Hashizume, Yoshio
AU - Murakami, Nobuyuki
PY - 2011/12
Y1 - 2011/12
N2 - 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.
AB - 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.
KW - Intravascular malignant lymphoma
KW - Large b-cell lymphoma
KW - Lymphomatosis cerebri
KW - Primary central nervous system lymphoma
KW - Progressive dementia
UR - http://www.scopus.com/inward/record.url?scp=80053572079&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=80053572079&partnerID=8YFLogxK
U2 - 10.1111/j.1440-1789.2011.01203.x
DO - 10.1111/j.1440-1789.2011.01203.x
M3 - Article
VL - 31
SP - 612
EP - 619
JO - Neuropathology
JF - Neuropathology
SN - 0919-6544
IS - 6
ER -