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