Primary cauda equina lymphoma confirmed by autopsy: A case report.

Compared with those involving the central nervous system, lymphomas involving the peripheral nervous system, namely neurolymphomatosis, are extremely rare. Neurolymphomatosis is classified as primary or secondary; the former is much rarer than the latter. Herein, we present an autopsied case of primary cauda equina lymphoma (PCEL), a type of primary neurolymphomatosis, with a literature review of autopsied cases of PCEL as well as primary neurolymphomatosis other than PCEL (non-PCEL primary neurolymphomatosis). A 70-year-old woman presented with difficulty walking, followed by paraplegia and then bladder and bowel disturbance. On magnetic resonance imaging, the cauda equina was diffusely enlarged and enhanced with gadolinium. The brainstem and cerebellum were also enhanced with gadolinium along their surface. The differential diagnosis of the patient included meningeal tumors (other than lymphomas), lymphomas, or sarcoidosis. The biopsy of the cauda equina was planned for a definite diagnosis, but because the patient deteriorated so rapidly, it was not performed. Eventually, she was affected by cranial nerve palsies. With the definite diagnosis being undetermined, the patient died approximately 1.5 years after the onset of disesase. At autopsy, the cauda equina was replaced by a bulky mass composed of atypical B-lymphoid cells, consistent with diffuse large B-cell lymphoma (DLBCL). The spinal cord was heavily infiltrated, as were the spinal/cranial nerves and subarachnoid space. There was metastasis in the left adrenal. The patient was finally diagnosed postmortem as PCEL with a DLBCL phenotype. To date, there have been a limited number of autopsied cases of PCEL and non-PCEL primary neurolymphomatosis (nine cases in all, including ours). The diagnosis is, without exception, B-cell lymphoma including DLBCL, and the histology features central nervous system parenchymal infiltration, nerve root involvement, and subarachnoid dissemination (lymphomatous meningitis). Metastases are not uncommon. All clinicians and pathologists should be aware of lymphomas primarily involving the peripheral nervous system.

COVID-19 associated Bell's Palsy and lumbosacral neurolymphomatosis in a patient with B-cell lymphoma-Case Report.

This is a case of acute onset unilateral Bell's Palsy during COVID-19 illness, coinciding with development of progressive leg pain, weakness, and sensation change. The patient was ultimately found to have a large B-cell lymphoma mass invading the sciatic nerve, lumbosacral plexus and the spinal canal with compression of cauda equina consistent with neurolymphomatosis. Although COVID-19 infection has been associated with Bell's palsy, Bell's palsy has also been reported with lymphoid malignancy. We review current literature on the association of Bell's palsy with COVID-19 infection and lymphoid malignancy, as well as review the diagnostic challenges of neurolymphomatosis. Providers should be aware of the possible association of Bell's palsy as harbinger of lymphoid malignancy.

Protean Manifestations and Diagnostic Challenges Including Discordance Between Electrodiagnostic-Radiologic Studies in Neurolymphomatosis.

INTRODUCTION: Malignant lymphocytic infiltration of the central nervous system (CNS) and peripheral nervous system (PNS) is diagnostically challenging and informs treatment and prognosis. CASE REPORT: We describe the clinical course of a 49-year-old man with CNS and PNS relapse of mantle cell lymphoma and the diagnostic modalities that enabled the diagnosis of neurolymphomatosis. CONCLUSION: This clinical phenotype reinforces previously reported presentations of neurolymphomatosis and the ability of multimodal diagnostics, when combined with clinical suspicion phenotype, to enable diagnosis of malignant lymphocytic infiltration of the CNS and PNS.

Neurolymphomatosis of the Brachial Plexus and its Branches: Case Series and Literature Review.

BACKGROUND: Neurolymphomatosis is a process of neoplastic endoneurial invasion, most strongly associated with non-Hodgkin's lymphoma. It must be distinguished from paraneoplastic, metabolic, nutritional and treatment-related causes of neuropathy that are common in this patient population. METHODS: This brief case series illustrates the protean manifestations of neurolymphomatosis of the brachial plexus, ranging from focal distal mononeuropathy to multifocal brachial plexopathy, either as the index manifestation of lymphoma or as a complication of relapsing disease. RESULTS: Prominent asymmetry, pain and nodular involvement on neuroimaging may help distinguish neurolymphomatosis from paraneoplastic immune demyelinating radiculoneuropathy. MR neurography criteria for the diagnosis of neurolymphomatosis include hyperintensity on T2 and STIR sequences, focal and diffuse nerve enlargement with fascicular disorganization and gadolinium enhancement. No specific anatomical distribution within the brachial plexus has, however, been found to be characteristic. Fluorodeoxyglucose-positron emission tomography (FDG-PET) imaging is the imaging modality with the highest sensitivity for detection of nodal or extranodal spread in lymphoma. CONCLUSIONS: Brachial plexus neuropathy in neurolymphomatosis is highly protean in its distribution, semiology and relation to lymphoma staging. Dedicated MRI and PET-CT imaging are leading diagnostic modalities.

Cauda equina syndrome secondary to neurolymphomatosis: Case report and literature review. / Síndrome de cauda equina secundario a neurolinfomatosis: presentación de un caso y revisión de la literatura.

Neurolymphomatosis is a rare disorder characterised by infiltration of neoplastic lymphocytes into the peripheral nervous system. A wide variety of symptoms can manifest depending on its nature and location, making its diagnosis a real challenge. Treatment is based on methotrexate, although various chemotherapy regimens are currently available for patients with systemic disease. We present the case of a male patient with neurolymphomatosis of the cauda equina, together with a review of all cases published to date.