scholarly journals Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

2021 ◽  
pp. 725-732
Author(s):  
Rojine Ariani ◽  
Leslie Ballas

Primary CNS extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma characterized predominantly by small B cells, plasma cells, monocytoid cells, and scattered large immunoblasts. As a slow-growing tumor that remains localized, primary CNS MZBL carries an excellent clinical prognosis. Here, we report two cases of primary CNS MZBL successfully treated using external beam radiation therapy along with a literature review.

2019 ◽  
Vol 104 (3) ◽  
pp. 357-362 ◽  
Author(s):  
Tine Gadegaard Hindsø ◽  
Bita Esmaeli ◽  
Frederik Holm ◽  
Lauge Hjorth Mikkelsen ◽  
Peter Kristian Rasmussen ◽  
...  

Backgrounds/aimsTo date, this is the largest cohort study on extranodal marginal zone B-cell lymphoma (EMZL) of the ocular adnexa (OA). The aim of the study was to characterise the clinical features of OA-EMZL.MethodsA retrospective multicentre study involving seven international eye cancer centres. Data were collected from 1 January 1980 through 31 December 2017. A total of 689 patients with OA-EMZL were included.ResultsThe median follow-up time was 42 months. The median age was 62 years (range, 8–100 years), and 55 % (378/689 patients) of patients were women. The majority of patients (82%, 558/680 patients) were diagnosed with primary OA-EMZL with Ann Arbor stage IE (90%, 485/541 patients) and American Joint Committee on Cancer stage T2 (61%, 340/557 patients) at the time of diagnosis. The orbit (66%, 452/689 patients) and the conjunctiva (37%, 255/689 patients) were the most frequently involved anatomical structures. The 5-year, 10-year and 20-year disease-specific survival (DSS) were 96%, 91% and 90%, respectively. Stage IE patients treated with external beam radiation therapy (EBRT) as monotherapy (10-year DSS, 95%) were found to have a better DSS than stage IE patients treated with chemotherapy (10-year DSS, 86%). Stage IIIE/IVE patients treated with chemotherapy and rituximab had a better DSS (10-year DSS, 96%) than stage IIIE/IVE patients treated with chemotherapy without rituximab (10-year DSS, 63%).Conclusions and relevanceEMZL is a slow-growing tumour with an excellent long-term survival. Low-dose EBRT as monotherapy should be considered in localised OA-EMZL. Rituximab-based chemotherapy should be chosen in those patients with disseminated disease.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4991-4991
Author(s):  
Ayad Al-Katib ◽  
Mary Mandziara ◽  
Susan Szpunar ◽  
Kleanthe Kolizeras ◽  
Mary Steigelman ◽  
...  

Abstract Abstract 4991 Anti-CD20 RIT is the most effective single agent treatment for B-cell lymphoma. However, it is not widely used in community-based oncology because of perceived complexity of treatment and toxicity. We analyzed the first 48 patients treated with RIT at our institution between November 2003 and February 2011. A work sheet for referral, screening and treatment process was developed jointly between Oncology and Nuclear Medicine and a mid-level provider (MM) was designated as RIT coordinator. Pre-treatment evaluation of all patients was according to recent consensus conference report on RIT (Witzig T et al 2011) including pretreatment imaging and bone marrow (BM) aspiration and biopsy. Unless dictated by patient's renal function, bladder control status and ability to comply with post treatment radiation safety requirement, the type of RIT (Ibritumomab Tiuxetan [Zevalin®] or Iodine 131 Tositumomab [Bexxar®]) was up to the discretion of referring oncologist. For Bexxar-treated patients, dosimetry imaging was done at 48 and 120 hours; Therapy dose, on day 8, was calculated to deliver 75 cGy to the total body. Zevalin imaging (In-111) was done at 48h and therapeutic dose of 0.4mCi/kg or 0.3mCi/kg depending on platelet counts (≥150K/μL or 100–149K/ μL, respectively) on days 7, 8, or 9. Patients were monitored for toxicity (NCI-CTC v4.03) and evaluated for response after 12 weeks of therapy according to Revised Response Criteria for Malignant Lymphoma (Cheson BD et al 2007). The SPSS v19.0 program was used for statistical analysis. Median age for the whole group was 60 at diagnosis (range 34–88) and 68.5 (37-89) at RIT. There were 19 males and 29 females. All patients, except one, had relapsed or refractory disease. Twenty two patients had diffuse large B-cell lymphoma (DLBCL) and 25 had follicular lymphoma (FL) with or without transformation. Thirty four patients were treated with Bexxar and 14 were treated with Zevalin. Thirty three of 45 evaluable patients for response achieved complete (CR n=20, 44%) or partial (PR, n=13, 29%) response for overall response rate (ORR) of 73%. There was lower, but statistically not significant, ORR for DLBCL (65%) compared with FL (79%). Other factors that did not influence ORR were race, gender, type of RIT, stage of disease and BM involvement by lymphoma. There was a trend towards association between IPI score and response in DLBCL (100% ORR for scores 0–1 vs 40% for score 3). FLIPI, however, was not predictive due to the high ORR in FL. Prior therapy and response to last regimen prior to RIT (LRPtoRIT) was predictive of ORR. 100%, 79% and 47% of patients who received 1, 2, ≥3 prior regimens responded to RIT, respectively (P 0.01). 84% of patients who achieved CR or PR to LRPtoRIT responded to RIT compared with 44% of those who did not respond to LRPtoRIT (P 0.014). 87% of patients who did not receive prior external beam radiation (RT) responded to RIT compared with 43% in those who were previously treated with RT (P 0.002). Finally, patients ≤60 years of age had higher response (93%) compared with those >60 (65%) (P 0.047). Multivariate analysis showed that response to LRPtoRIT was still significant even after adjusting for age and RT prior to RIT. From Kaplan-Meier analysis, the median survival of the whole group was 48 months; 39 months for DLBCL and 82 months for FL (P 0.096). Patients who responded to RIT had a median survival of 81 months compared with those who did not (4.2 months) (P 0.000). Other factors that predicted for survival in univariate analysis were: number of prior regimens (P 0.017), response to LRPtoRIT (P 0.001), and prior external beam radiation (P 0.024). In multivariate analysis, response to RIT and response to LRPtoRIT were significant predictors of survival (P 0.048 and 0.026, respectively). There were no treatment-related deaths. Grade 3/4 toxicity was only hematologic including thrombocytopenia (38%), neutropenia (31%) and anemia (12.5%). Median time to nadir was 5, 6 and 8 weeks post therapy for platelets, neutrophils and hemoglobin, respectively. Two patients developed secondary AML and one patient developed MDS, all with complex chromosomal abnormalities. We conclude that with coordinated effort, RIT can be safely and effectively delivered in routine community setting with results comparable to those reported in clinical trials. Prior therapy and response to LRPtoRIT predict response to RIT whereas response to RIT and response to LRPtoRIT are predictive of overall survival. Disclosures: No relevant conflicts of interest to declare.


Author(s):  
Adrian J. Rodrigues ◽  
Michael C. Jin ◽  
Adela Wu ◽  
Hriday P. Bhambhvani ◽  
Gordon Li ◽  
...  

OBJECTIVE Although past studies have associated external-beam radiation therapy (EBRT) with higher incidences of secondary neoplasms (SNs), its effect on SN development from pediatric low-grade gliomas (LGGs), defined as WHO grade I and II gliomas of astrocytic or oligodendrocytic origin, is not well understood. Utilizing a national cancer registry, the authors sought to characterize the risk of SN development after EBRT treatment of pediatric LGG. METHODS A total of 1245 pediatric patient (aged 0–17 years) records from 1973 to 2015 were assembled from the Surveillance, Epidemiology, and End Results (SEER) database. Univariable and multivariable subdistribution hazard regression models were used to evaluate the prognostic impact of demographic, tumor, and treatment-related covariates. Propensity score matching was used to balance baseline characteristics. Cumulative incidence analyses measured the time to, and rate of, SN development, stratified by receipt of EBRT and controlled for competing mortality risk. The Fine and Gray semiparametric model was used to estimate future SN risk in EBRT- and non–EBRT-treated pediatric patients. RESULTS In this study, 366 patients received EBRT and 879 did not. Forty-six patients developed SNs after an LGG diagnosis, and 27 of these patients received EBRT (OR 3.61, 95% CI 1.90–6.95; p < 0.001). For patients alive 30 years from the initial LGG diagnosis, the absolute risk of SN development in the EBRT-treated cohort was 12.61% (95% CI 8.31–13.00) compared with 4.99% (95% CI 4.38–12.23) in the non–EBRT-treated cohort (p = 0.013). Cumulative incidence curves that were adjusted for competing events still demonstrated higher rates of SN development in the EBRT-treated patients with LGGs. After matching across available covariates and again adjusting for the competing risk of mortality, a clear association between EBRT and SN development remained (subhazard ratio 2.26, 95% CI 1.21–4.20; p = 0.010). CONCLUSIONS Radiation therapy was associated with an increased risk of future SNs for pediatric patients surviving LGGs. These data suggest that the long-term implications of EBRT should be considered when making treatment decisions for this patient population


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5266-5266
Author(s):  
Emily Harris ◽  
Ariel Sindel ◽  
Ian McConnell ◽  
William Clark ◽  
Alden Chesney ◽  
...  

Introduction Primary cutaneous B-cell lymphomas (PCBCLs) arise within the skin without evidence of extracutaneous involvement at the time of diagnosis. The incidence of PCBCL is rare, and PCBCLs are less common than primary cutaneous T-cell lymphomas. There are three subgroups of PCBCL: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle-cell lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). The 5-year survival rates of the former two subtypes exceed 90%, whereas PCDLBCL-LT is approximately 50% (Wilcox 2015). We describe a rare case of indolent PCMZL transforming into the more aggressive PCDLBCL-LT as well as a review of literature and case series. Case A 62-year-old man with history of PCMZL refractory to external beam radiation and rituximab chemotherapy presented with a two-month history of an enlarging rash and three nodules on his left leg. On physical exam, two violaceous, infiltrative plaques with distinct borders were present on the left leg, resembling his past PCMZL. Three new lesions were described as deep-seated 2 cm erythematous nodules (Fig 1). Histologic review of the new nodules revealed a diffuse, dermal-based atypical lymphoid infiltrate extending from the papillary dermis into the subcutis. The atypical lymphoid cells contained mostly round nuclei, vesicular chromatin, and prominent nucleoli consistent with an immunoblastic and centroblastic morphology (Fig 2). They expressed B-cell markers such as CD79a and CD19, and were negative for CD20 most likely due to prior treatment with rituximab. Immunohistochemistry demonstrated positivity for Bcl-2 and MUM1. Discussion Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a rare and aggressive B-cell lymphoma. This subtype is positive for CD20 and CD79, negative for CD5 and CD10, and strongly positive for Bcl-2 and MUM1. It has the gene expression profile of the ABC subtype. A nodule on the lower leg is an ominous sign given this subtype's predilection for lower extremities. This subtype affects the elderly as opposed to the indolent subtypes that arise in middle age (Willemze 2005). Prognosticating PCDLBCL-LT is challenging, as survival rates vary from 40 to 70% (Willemze 2005). Additionally, the aggressive nature of PCDLBCL-LT confers use of more aggressive, systemic chemotherapy regimens. Rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone (R-CHOP) chemotherapy followed by external beam irradiation is recommended, though no large prospective clinical trials have been performed to date. If lesions remain refractory to R-CHOP, the treatment goal should be redirected to treat as a refractory systemic diffuse large B-cell lymphoma which may require bone marrow transplant or targeted agents with known activity in the ABC subtype such as lenalidomide or ibrutinib. Transformation from PCMZL to PCDLBCL-LT is a rare event and has only been described in 2 case series (Table 1). In our review of the prior cases, the age group was heterogenous ranging from 29 years-old to 80 years-old. There was no consensus treatment, however, the majority of patients did receive at least CHOP-based regimen. Survival ranged from 24 months to 180 months, showing the heterogeneity of the disease process. In our patient, he underwent R-CHOP but progressed and then received salvage R-ICE with CR. He then underwent bone marrow transplantation but unfortunately had recurrence 36 days post-transplantation, proven PCDLBCL-LT, and he died 161 days following his allogeneic transplant. Conclusion Although PCMZL and PCFCL frequently recur despite treatment, transformation to PCDLBCL-LT is unusual. To date, transformation from PCMZL to PCDLBCL-LT has been described rarely in the literature (Table 1). PCMZL is indolent and is treated with external beam radiation therapy or surgical excision with excellent prognosis, the aggressive PCDLBCL-LT subtype has a poor outcome and requires systemic chemotherapy. Because of the vast difference in prognosis and treatment between PCMZL and PCDLBCL-LT, early recognition and exhaustive work-up is beneficial to the patient. Considering this transformation is both rare and diagnostically challenging, clinicians should have heightened concern for new nodular lesions in the setting of recurrent indolent primary cutaneous B-cell lymphomas. Disclosures Yazbeck: Celgene: Membership on an entity's Board of Directors or advisory committees; Gilead Sciences: Research Funding; Seattle Genetics: Membership on an entity's Board of Directors or advisory committees.


10.37206/88 ◽  
2005 ◽  
Author(s):  
Ellen Yorke ◽  
Rodica Alecu ◽  
Li Ding ◽  
Doracy Fontenla ◽  
Andre Kalend ◽  
...  

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