Project Title

Recurrent Dural based Extra Nodal marginal Zone Lymphoma of Central Nervous System

Authors' Affiliations

Chandana Kamireddy, Division of Oncology/Hematology, Department of Medicine, Quillen College of Medicine, East Tennessee State University, Johnson City, TN Parisa Vahhabaghai, Department of pathology, Quillen College of Medicine, East Tennessee State University, Johnson City, TN Kanishka Chakraborty, Division of Oncology/Hematology, Department of Medicine, Quillen College of Medicine, East Tennessee State University, Johnson City, TN Devapiran Jaishankar, Division of Oncology/Hematology, Department of Medicine, Quillen College of Medicine, East Tennessee State University, Johnson City, TN

Location

Culp Ballroom

Start Date

4-7-2022 9:00 AM

End Date

4-7-2022 12:00 PM

Poster Number

39

Faculty Sponsor’s Department

Other - please list

Medical Oncology

Name of Project's Faculty Sponsor

Devapiran Jaishankar

Classification of First Author

Medical Resident or Clinical Fellow

Competition Type

Non-Competitive

Type

Poster Case Study Presentation

Project's Category

Lymphomas

Abstract or Artist's Statement

Marginal zone B-cell lymphomas (MZBL) constitute 7% of all non-Hodgkin lymphomas, being the third most common subtype after diffuse large B-cell lymphoma(DLBCL) and follicular lymphoma. Extranodal MZBL (ENMZBL) most commonly arise from the mucosa-associated lymphoid tissue (MALT lymphoma), with stomach being the most common site. ENMZBL involving the dura is anatomically unusual and very rare.

A 54 year old female presented to the hospital with worsening headaches and new onset generalized tonic clonic seizures. Complete blood counts and chemistry were unremarkable. No constitutional symptoms, new / progressive lymphadenopathy reported. Magnetic resonance Imaging( MRI) brain showed an enhancing right subdural soft tissue lesion overlying the right frontotemporal lobe suggestive of meningioma versus metastasis. Computed Tomography (CT) chest/abdomen/pelvis revealed no mass or lymphadenopathy. Lumbar Puncture cerebrospinal fluid cytology was negative for malignancy. She underwent brain biopsy. Pathology revealed diffuse infiltrate of small to medium-sized lymphoid cells, Immunohistochemical stains positive for CD 20, PAX5, CD 79a, Ki-67 at 5-10%, weakly positive for MUM1 and BCL2, negative for CD3, CD5, CD10, BCL6, cyclin D1, consistent with ENMZBL. Bone marrow biopsy and aspiration negative for involvement with lymphoma. Patient received local/regional therapy with radiation (XRT), total dose 24 Gy in 12 fractions. She presented six months later with worsening neck pain. MRI cervical spine revealed diffuse thick dural based enhancement within the spinal canal at C1-C4 levels leading to moderate-to-severe spinal canal stenosis at C2-C3 level with significant soft tissue extension. Repeat labs, systemic imaging, and bone marrow biopsy continued to show no evidence of systemic disease. She received low dose XRT to the entire craniospinal axis (dose-4Gy). Patient developed profound pancytopenia secondary to craniospinal XRT. After count recovery she initiated daily oral Ibrutinib (560 mg) with plans for a treatment duration of one year.

Dural based ENMZL usually present as solitary masses, mimicking meningioma's. Marked female predilection is seen with median age of onset 50 years. Very few cases have been reported in literature with no standard therapy being described. ENMZL are usually indolent requiring less aggressive therapy. In contrast, primary CNS lymphoma (PCNSL) of diffuse large B cell histology is usually aggressive with high tendency to relapse, requiring treatment with high dose methotrexate based regimes. Dural based ENMZL therapy entails local treatments such as surgery and radiation therapy (relatively low dose radiation usually effective with prolonged durable responses). Systemic treatment with single agent Rituximab or with Tyrosine Kinase inhibitors like Ibrutinib with CNS penetration can also be considered. Long-term follow-up is recommended even in those patients who achieved complete remission as relapses may occur.

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Apr 7th, 9:00 AM Apr 7th, 12:00 PM

Recurrent Dural based Extra Nodal marginal Zone Lymphoma of Central Nervous System

Culp Ballroom

Marginal zone B-cell lymphomas (MZBL) constitute 7% of all non-Hodgkin lymphomas, being the third most common subtype after diffuse large B-cell lymphoma(DLBCL) and follicular lymphoma. Extranodal MZBL (ENMZBL) most commonly arise from the mucosa-associated lymphoid tissue (MALT lymphoma), with stomach being the most common site. ENMZBL involving the dura is anatomically unusual and very rare.

A 54 year old female presented to the hospital with worsening headaches and new onset generalized tonic clonic seizures. Complete blood counts and chemistry were unremarkable. No constitutional symptoms, new / progressive lymphadenopathy reported. Magnetic resonance Imaging( MRI) brain showed an enhancing right subdural soft tissue lesion overlying the right frontotemporal lobe suggestive of meningioma versus metastasis. Computed Tomography (CT) chest/abdomen/pelvis revealed no mass or lymphadenopathy. Lumbar Puncture cerebrospinal fluid cytology was negative for malignancy. She underwent brain biopsy. Pathology revealed diffuse infiltrate of small to medium-sized lymphoid cells, Immunohistochemical stains positive for CD 20, PAX5, CD 79a, Ki-67 at 5-10%, weakly positive for MUM1 and BCL2, negative for CD3, CD5, CD10, BCL6, cyclin D1, consistent with ENMZBL. Bone marrow biopsy and aspiration negative for involvement with lymphoma. Patient received local/regional therapy with radiation (XRT), total dose 24 Gy in 12 fractions. She presented six months later with worsening neck pain. MRI cervical spine revealed diffuse thick dural based enhancement within the spinal canal at C1-C4 levels leading to moderate-to-severe spinal canal stenosis at C2-C3 level with significant soft tissue extension. Repeat labs, systemic imaging, and bone marrow biopsy continued to show no evidence of systemic disease. She received low dose XRT to the entire craniospinal axis (dose-4Gy). Patient developed profound pancytopenia secondary to craniospinal XRT. After count recovery she initiated daily oral Ibrutinib (560 mg) with plans for a treatment duration of one year.

Dural based ENMZL usually present as solitary masses, mimicking meningioma's. Marked female predilection is seen with median age of onset 50 years. Very few cases have been reported in literature with no standard therapy being described. ENMZL are usually indolent requiring less aggressive therapy. In contrast, primary CNS lymphoma (PCNSL) of diffuse large B cell histology is usually aggressive with high tendency to relapse, requiring treatment with high dose methotrexate based regimes. Dural based ENMZL therapy entails local treatments such as surgery and radiation therapy (relatively low dose radiation usually effective with prolonged durable responses). Systemic treatment with single agent Rituximab or with Tyrosine Kinase inhibitors like Ibrutinib with CNS penetration can also be considered. Long-term follow-up is recommended even in those patients who achieved complete remission as relapses may occur.