Bing-Neel syndrome presenting as isolated CNS lymphoplasmacytic lymphoma: A case report and review of the literature

Grainger, Brian; Issa, Samar

doi:10.1016/j.jocn.2019.11.013

Cited by 5 publications

(4 citation statements)

References 13 publications

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“…[1][2][3] Rarely, BNS precedes the diagnosis of WM and appears as a primary CNS LPL, having only been described in twenty-four cases. 7 Prognosis appears to be better in these cases as compared to those with a prior history of WM whose disease progresses to BNS. 3 The evaluation for BNS begins with diagnostic testing including gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and whole spine as well as cerebrospinal fluid (CSF) sampling for cytology, flow cytometry, and mutational analyses.…”

Section: Discussionmentioning

confidence: 85%

Primary central nervous system diffuse large B‐cell lymphoma masqueraded as Bing‐Neel syndrome: Steps in management and review of future directions

Delasos

Phachu

Shetty

et al. 2021

Clinical Case Reports

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Section: Discussionmentioning

confidence: 85%

Primary central nervous system diffuse large B‐cell lymphoma masqueraded as Bing‐Neel syndrome: Steps in management and review of future directions

Delasos

Phachu

Shetty

et al. 2021

Clinical Case Reports

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“…It is also noted that Bing-Neel syndrome can be diagnosed without fulfilling WM diagnostic criteria. 18 …”

Section: Discussionmentioning

confidence: 99%

“…It is also noted that Bing-Neel syndrome can be diagnosed without fulfilling WM diagnostic criteria. 18 There is still no consensus guideline for optimal treatment for patients with primary CNS LPL. The chosen therapies in reported cases included tumor resection, chemotherapy, radiation therapy, and targeted therapy.…”

Section: Discussionmentioning

confidence: 99%

External-Beam Radiotherapy Alone Management of Primary CNS Lymphoplasmacytic Lymphoma: A Vietnamese Case Report and Literature Review

Van¹,

Van²,

Van³

et al. 2021

JBM

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Background Primary central nervous system (CNS) lymphoma is an uncommon non-Hodgkin disease limited to the CNS, and most cases are diffuse large B-cell lymphomas. Other pathologies, including lymphoplasmacytic lymphoma (LPL), are exceedingly rare and poorly understood. The clinical presentation of primary CNS LPL is diverse. It depends on the original site and the tumor’s extension. There is currently no consensus on a treatment strategy for this uncommon manifestation. To our knowledge, no previously published case was successfully treated with radiation therapy alone. Case Presentation We present here a case of primary CNS LPL. A 46-year-old, previously healthy woman was presented with a worsening headache and lower extremity numbness. Multifocal enhanced masses were detected in an MRI with biopsy results consistent with LPL. A complete staging workup was performed with no evidence of systemic disease. The patient received external-beam radiotherapy alone and had a complete remission. After 2 years of follow-up, she remains disease-free. Conclusion Radiation alone is a promising treatment option for primary CNS lymphoplasmacytic lymphoma.

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“…Most patients with BNS will be diagnosed at the time of disease progression of pre-existing WM. Rarer are those patients who develop neurologic symptoms as an initial manifestation of WM [ 4 , 5 ]. The most common symptoms include gait disturbances (12–48%), motor limb deficits (14–35%), and cranial nerve palsies (29–35%) of the facial and oculomotor nerves [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

Mixed Diffuse and Tumoral Form of Bing-Neel Syndrome Successfully Treated with Ibrutinib

Naveed Ahmad,

Schroeder,

Yun

et al. 2023

Case Rep Oncol

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Introduction: Bing-Neel syndrome (BNS) is a rare and heterogenous manifestation of Waldenström macroglobulinemia (WM) involving central nervous system (CNS) infiltration by malignant lymphoplasmacytic cells. Efforts to standardize diagnostic criteria have improved in recent years, as have treatment options including the use of the Bruton tyrosine kinase inhibitor (BTKI) ibrutinib. Case Presentation: Here, we present the case of a 70-year-old male with a remote history of WM previously treated with bendamustine and rituximab, who presented to medical attention with several months of left-sided weakness, headache, and ataxia. Brain magnetic resonance imaging revealed numerous enhancing masses in the bilateral cerebral hemispheres, inferior medulla, and upper cervical spine. Laboratory studies showed serum IgM lambda monoclonal gammopathy and elevated free serum kappa and lambda light chains, while cerebrospinal fluid flow cytometry revealed CD19+ B cells. Stereotactic brain biopsy of a right frontal brain lesion was consistent with lymphoplasmacytic lymphoma, confirmed by a positive MYD88 L265P mutation. He received ibrutinib 420 mg orally daily, and this resulted in appreciable clinical and radiologic responses, which have persisted over a 31-month period. Conclusion: The advent of molecularly targeted agents and novel therapies for WM has provided patients and clinicians with additional therapeutic options. The use of BTK inhibitors with their high-level CNS penetrance, in particular, offers a novel way to treat BNS and improve patient overall survival while maintaining a high level of quality of life. We discuss the importance of MYD88 L265P testing in the context of BNS as well as the expanding role of BTKIs in treating this disease.

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Bing-Neel syndrome presenting as isolated CNS lymphoplasmacytic lymphoma: A case report and review of the literature

Cited by 5 publications

References 13 publications

Primary central nervous system diffuse large B‐cell lymphoma masqueraded as Bing‐Neel syndrome: Steps in management and review of future directions

Primary central nervous system diffuse large B‐cell lymphoma masqueraded as Bing‐Neel syndrome: Steps in management and review of future directions

External-Beam Radiotherapy Alone Management of Primary CNS Lymphoplasmacytic Lymphoma: A Vietnamese Case Report and Literature Review

Mixed Diffuse and Tumoral Form of Bing-Neel Syndrome Successfully Treated with Ibrutinib

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