Primary cauda equina lymphoma confirmed by autopsy: A case report
Keisuke Ishizawa1,2, Takashi Komori2, Rui Shimazaki3
1Department of Pathology, Saitama Medical University, Moroyama, Japan.
Summary
Primary cauda equina lymphoma (PCEL) is an extremely rare peripheral nervous system lymphoma. Autopsy confirmed PCEL with diffuse large B-cell lymphoma (DLBCL) phenotype, highlighting the need for awareness among clinicians and pathologists.
Area of Science:
- Neuropathology
- Oncology
- Peripheral Nervous System Disorders
Background:
- Lymphomas involving the peripheral nervous system (neurolymphomatosis) are rare compared to central nervous system lymphomas.
- Primary neurolymphomatosis, originating in the peripheral nervous system, is significantly rarer than secondary involvement.
Approach:
- Presents an autopsied case of primary cauda equina lymphoma (PCEL), a rare form of primary neurolymphomatosis.
- Includes a literature review of autopsied cases of PCEL and other primary neurolymphomatosis (non-PCEL).
Key Points:
- The case involved a 70-year-old woman with progressive neurological deficits, including paraplegia and cranial nerve palsies.
- Postmortem autopsy revealed diffuse large B-cell lymphoma (DLBCL) replacing the cauda equina, with spinal cord and nerve infiltration.
- Reviewed literature identified nine autopsied cases of PCEL and non-PCEL primary neurolymphomatosis, all diagnosed as B-cell lymphoma (including DLBCL).
Conclusions:
- Primary neurolymphomatosis, particularly PCEL, is invariably a B-cell lymphoma, often DLBCL.
- Histological features include central nervous system parenchymal infiltration, nerve root involvement, and lymphomatous meningitis.
- Metastases are common, and increased awareness among clinicians and pathologists is crucial for diagnosis and management.
