Characteristics of primary extranodal marginal zone B-cell lymphoma in Korea: conjunctiva versus other ocular adnexa

AimsTo compare the clinical characteristics and prognosis of primary conjunctival versus other ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL).MethodsRetrospective review of clinical records for all consecutive patients with primary ocular adnexal EMZL treated from March 1995 to December 2015.Results198 patients were evaluated including 81 with primary conjunctival and 117 with other ocular adnexal EMZL. Conjunctival EMZL was found at a younger age (40.3±10.3vs 54.3±13.4 years, p<0.0001) with a female predilection (75.3%vs35.9%, p<0.0001) and had a higher rate of bilaterality (40.7%vs11.1%, p<0.0001) and a lower rate of systemic involvement (1.2%vs9.4%, p=0.030) compared with other ocular adnexal EMZLs. Conjunctival EMZL also showed a higher rate of complete response to primary treatment (98.8%vs89.5%, p=0.016) than other ocular adnexal EMZLs; however, recurrence and lymphoma-related death rates were not different between the two groups (p>0.05). Kaplan-Meier estimates for disease-specific survival at 5 and 10 years were 98.2% and 98.2% in conjunctival and 98.6%, respectively, and 95.2% in other ocular adnexal EMZLs. Univariate analysis showed that systemic involvement was negatively associated with conjunctival tumour location and positively associated with age (OR=0.35 and OR=1.05, p=0.045 and p=0.012, respectively), and treatment response was positively associated with conjunctival tumour location and negatively associated with age (OR=3.02 and OR=0.95, p=0.035 and p=0.009, respectively).ConclusionsConjunctival EMZL shows unique demographic characteristic compared with other ocular adnexal EMZLs. Long-term follow-up is required due to late recurrence in ocular adnexal EMZL.

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Ctdna Monitoring in Predicting Relapse of Primary Mediastinal B-Cell Lymphoma

Blood ◽

10.1182/blood-2020-142349 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 36-36

Author(s):

Diwen Pang ◽

Xinmiao Jiang ◽

Ling Huang ◽

Yan Teng ◽

Sichu Liu ◽

...

Keyword(s):

B Cell ◽

Signaling Pathway ◽

Conflicts Of Interest ◽

Cell Lymphoma ◽

Univariate Analysis ◽

B Cell Lymphoma ◽

Complete Response ◽

Genetic Sequencing ◽

Dismal Prognosis ◽

Prediction Of Relapse

Background: Primary mediastinal B-cell lymphoma (PMBCL) is a rare subtype of aggressive B-cell lymphoma. The majority of relapses occur within the first few months resulting in a dismal prognosis, thus we need to identify the risk of early relapse. Circulating tumor DNA (ctDNA)-based response assessment may enable response adapted therapy and early prognostication. In this study, we aim to clarify the role of ctDNA-monitoring in early prediction of relapse. Methods: We retrospectively analyzed the characteristics of 38 patients with PMBCL. The genetic sequencing and ctDNA monitoring were conducted by target next-generation sequencing (NGS). Results: The median age was 32 years old and 26 (68.4%) were female. Most cases (31/38, 81.6%) were diagnosed with stage I or II disease and fourteen (36.8%) cases present with extranodal involvement. The 5-year PFS and OS for PMBCL patients were 65.7 and 72.1%, respectively. High IPI score were associated with poor survival by univariate analysis. The complete response (CR) rate and overall response rate of R-CHOP was similar to R-EPOCH (69.2% vs. 70.0%, P =1.000 and 84.6% vs. 95.0%, P =0.547, respectively), however the 5-year PFS and OS rate for R-EPOCH seems longer than those for the R-CHOP (PFS 82.9% vs. 53.8%, P=0.0569; OS 92.9% vs. 60.6%, P=0.0567). Radiotherapy was less delivered in the patients who received R-EPOCH than R-CHOP (5.0% vs.53.8%, P=0.003) and had no impact on prognosis. We performed genetic sequencing on twenty-three cases. STAT6(15/23, 65.2%), SOCS1(13/23, 56.5%), TNFAIP3(13/23, 56.5%) were the most common affected genes in both response and refractory/relapsed patients. Cell cycle, FoxO signaling pathway and TNF signaling pathway were more frequently involved in refractory patients. ctDNA monitoring was conducted in 16 cases and fifteen cases show undetectable ctDNA after a median of 2 (range from 1 to 5) cycles and one refractory case presented with durable positive ctDNA. Among 4 relapse cases, 3 had prior detectable ctDNA half a month earlier before imaging manifestations of relapse. Regarding prediction of relapse, positive predictive value of ctDNA is 100%. Conclusions: Intensive induction chemotherapy can improve prognosis of PMBCL and ctDNA precisely predicted relapse. As most cases relapse within a few months, ctDNA-monitoring is crucial to improve prognosis. Disclosures No relevant conflicts of interest to declare.

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NIMG-56. THE RARE CASE OF PRIMARY DURAL BASED MARGINAL ZONE B-CELL LYMPHOMA CAMOUFLAGING AS MILD FOCAL DURA THICKNESS AND PACYMENINGEAL ENHANCEMENT

Neuro-Oncology ◽

10.1093/neuonc/noaa215.669 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii160-ii160

Author(s):

Maya Hrachova ◽

Mari Perez-Rosendahl ◽

Xiao-Tang Kong

Keyword(s):

B Cell ◽

Marginal Zone ◽

Cell Lymphoma ◽

Bone Biopsy ◽

Cranial Nerves ◽

Malignant Neoplasm ◽

Brain Biopsy ◽

B Cell Lymphoma ◽

Radiographic Findings ◽

Systemic Involvement

Abstract INTRODUCTION Primary Dural Based Marginal Zone B-Cell Lymphoma (MZBCL) is a subtype of PCNSL with an incidence rate ranging from 0.6 to 3% of all brain tumors. MZBCL typically presents as an extra-axial mass resembling meningioma. Here we report an unusual case of MZBCL with initial radiographic findings of mild nonspecific thickening tentorium and pachymeningeal enhancement surrounding 7th and 8th cranial nerves. CASE REPORT A 58-year old woman with clinical history of CMV infection, polyclonal gammopathy and unruptured left ICA aneurysm post coil embolization who presented for an evaluation of mild thickening and enhancement of the left tentorium cerebelli and 7th/8th nerve root complex. Differential considerations included inflammatory/autoimmune conditions (idiopathic hypertrophic pachymeningitis, neurosarcoidosis, Tolosa-Hunt syndrome), infections, structural lesion, benign or malignant neoplasm. Serum studies were normal. Multiple CSF studies were negative. Flow cytometry showed no malignant cells with few small lymphocytes. She was followed with the imaging surveillance for nine months until further increased thickening of dura on MRI. She underwent cerebellar dural biopsy that was consistent with MZBCL composed mainly of small CD20+ B-cells and negative MYD88. PET scan showed no systemic involvement. Bone biopsy revealed no evidence of lymphoma. The focal leptomeningeal enhancement improved significantly after she received four doses of systemic rituximab treatments. DISCUSSION Our case highlights the importance of surveillance and brain biopsy in cases of mild focal dura/pachymeningeal thickness and enhancement if no conclusive diagnosis has been established, as it might be one of the rare tumors such as MZBLC.

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