Neurolymphomatosis of multifocal peripheral nerve involvement: a case report.

The infiltration and invasion of nerve trunks, nerve roots, and cranial nerves by lymphomatous malignant cells is defined as "neurolymphomatosis". It is mainly caused by lymphoma cells directly infiltrating the peripheral nerves, with a low incidence. Neurolymphomatosis is a rare condition of neoplastic endoneurial invasion, which is primary or secondary to non-Hodgkin's lymphoma and leukemia. We describe a case of primary peripheral neurolymphomatosis of multifocal involvement in a 77-year-old male patient. He presented with left lower limb pain and was diagnosed with CD20+ diffuse large B cell lymphoma (DLBCL). Magnetic resonance imaging (MRI), fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) computed tomography (CT), and nerve biopsy contributed to the diagnosis. Genomic profiling, programmed death ligand-1 (PD-L1) expression and tumor mutational burden (TMB) were also assessed. CDKN2A/CDKN2B deletions have been identified. PD-L1 expression assessed by 28-8 antibody was 1% positivity, and TMB of the sample was 11.6 muts/Mb. The patient responded well to rituximab combined with chemotherapy, however, he died after 3 cycles of chemotherapy due to severe lung infection and subsequent complication of respiratory failure. Here we report the clinical, radiological, pathological and molecular findings of the patient affected by multifocal neurolymphomatosis without systemic involvement of other organs.

Magnetic Resonance Imaging-negative, Rituximab-resistant Neurolymphomatosis as a Paradoxical Presentation of Relapsed Primary Adrenal Lymphoma.

Primary adrenal lymphoma (PAL) is rare and known to have a predilection for central nervous system (CNS) relapse. A 70-year-old man with a 2-year history of primary aldosteronism presented because of a fever. He was hypotensive, and his adrenal glands were unequivocally enlarged. PAL was diagnosed. Despite showing an initial response to immunochemotherapy, progressive paralysis ensued. Magnetic resonance imaging findings were negative, and rituximab was ineffective. His debilitated condition hindered further chemotherapy. A postmortem examination revealed lymphoma relapse in the systemic peripheral nerves. The sequential presentation of two rare lymphomas implies that PAL might have a predilection for not only the CNS but also peripheral nerves.

Neurolymphomatosis of the Sciatic and Tibial Nerves.

Neurolymphomatosis (NL) is an uncommon clinical condition, characterised by lymphomatous infiltration of the central and/or peripheral nervous system. Most often it is caused by B-cell non-Hodgkin's lymphoma (NHL). Clinically, patients usually present with neuropathy involving the nerve roots, plexuses, peripheral or cranial nerves. NL usually occurs as a complication of prior lymphoma, but it can also present in the form of relapsed lymphoma. It is important to diagnose and start early treatment in all cases of nodal or visceral (including neural) lymphoma with chemo and/or radiation therapy. The PET-CT and MRI can help in making diagnosis. We are presenting a case of 28-year male patient, diagnosed as diffuse large B-cell lymphoma on the background of follicular lymphoma, which initially responded to treatment but then presented with NL, based on clinical history and radiological findings which were confirmed by histopathology.

Neurolinfomatosis primaria de cola de caballo como manifestación inicial del virus de la inmunodeficiencia humana / Primary neurolymphomatosis in the cauda equina as the initial symptom of human immunodeficiency virus

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[Primary neurolymphomatosis in the cauda equina as the initial symptom of human immunodeficiency virus]. / Neurolinfomatosis primaria de cola de caballo como manifestacion inicial del virus de la inmunodeficiencia humana.

TITLE: Neurolinfomatosis primaria de cola de caballo como manifestacion inicial del virus de la inmunodeficiencia humana.

B-cell peripheral neurolymphomatosis: MRI and 18F-FDG PET/CT imaging characteristics.

OBJECTIVE: To examine the MRI and 18F-FDG PET/CT imaging characteristics of peripheral neurolymphomatosis. MATERIALS AND METHODS: All institutional cases of neurolymphomatosis with an MRI or 18F-FDG PET/CT from 2000 to 2017 were retrospectively reviewed. Included cases were biopsy-proven neurolymphomatosis or lymphoma patients with clinical and imaging evidence of neurolymphomatosis that resolved after chemotherapy. Multiple imaging parameters and clinical characteristics were recorded. RESULTS: There were 27 cases of B-cell neurolymphomatosis in 25 patients (18 M, 7 F; mean age 64.6 ± 10.0 years). Of the total cases, 85% (23/27) were biopsy-proven. Most were diagnosed after disease progression or recurrence (20/27, 74%), and presented with isolated nerve involvement (18/27, 67%). Bone marrow biopsy (17/19, 89%) and CSF cytology (16/23, 70%) were usually negative. On 18F-FDG PET/CT, neurolymphomatosis presented as a linear or fusiform (23/26, 88%), FDG-avid (average SUVmax: 7.1 ± 4.5, range, 1.5-17.0) mass, and on MRI as a T2-weighted hyperintense (21/22, 95%), enhancing (21/22, 95%), linear or fusiform mass (19/22, 86%), with associated muscle denervation (14/22, 64%). FDG avidity was significantly higher in patients with muscular denervation on MRI (mean SUVmax 8.2 ± 4.6 vs. 4.3 ± 2.3, p = 0.04). CONCLUSIONS: B-cell neurolymphomatosis most commonly manifests as T2-weighted hyperintense, enhancing linear or fusiform neural enlargement associated with muscular denervation on MRI, with intense FDG activity on PET/CT. It is most often an isolated site of disease, presenting after progression or recurrence. A familiarity with the imaging appearance of neurolymphomatosis can help refine the differential diagnosis, direct biopsy, and aid in accurate diagnosis.

Limited efficacy of high-dose methotrexate in patients with neurolymphomatosis.

Neurolymphomatosis (NL) is a rare manifestation of non-Hodgkin lymphoma, in which malignant cells infiltrate the peripheral nerves. Most patients are treated with high-dose methotrexate (HD-MTX)-based systemic chemotherapy regimens similar to patients with central nervous system lymphoma. However, because NL is rare, the efficacy of HD-MTX is largely unknown. We reviewed medical records of patients diagnosed with NL over the past 10 years and identified 18 patients. The underlying hematological malignancy was diffuse large B-cell lymphoma (DLBCL) in 10 patients (55.6%), intravascular large B-cell lymphoma in six (33.3%), and other types in two patients. Ten patients were treated with HD-MTX-based systemic chemotherapy; the response rates with and without HD-MTX-based chemotherapy were 100% (n = 10) and 85.7% (n = 6), respectively (P = 0.41). The median progression-free and overall survival rates of patients with versus without HD-MTX treatment were 6.4 vs. 8.5 months (P = 0.97) and 13.5 vs. 8.5 months (P = 0.63), respectively. Despite the initial favorable responses, rapid disease recurrence was observed in most patients administered HD-MTX-based chemotherapy. Our observations suggest that HD-MTX-based chemotherapy may have insufficient efficacy against NL, and that other therapeutic approaches are required to improve the outcomes of patients with this rare disease.

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.