Primary pancreatic lymphoma: A population-based analysis using the Surveillance, Epidemiology and End Results database.

2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 303-303
Author(s):  
M. V. Mishra ◽  
S. W. Keith ◽  
X. Shen ◽  
T. Biswas

303 Background: Primary pancreatic lymphoma (PPL) is a rare disease, accounting for only 0.5% of all pancreatic masses. The clinical presentation of PPL is similar to that of pancreatic adenocarcinoma and the two are difficult to distinguish radiographically. A paucity of literature exists on the epidemiology and outcomes of PPL. Given the limited case reports published on PPL, a comparison of the different treatment modalities has previously not been possible. Here, we present a series of 523 cases of PPL obtained from the Surveillance, Epidemiology, and End Results (SEER) database to investigate the tumor characteristics and compare the different treatment modalities. Methods: Patients diagnosed with a primary pancreatic lymphoma from 1973-2007 were identified. Data on patient and tumor characteristics as well as initial treatment with surgery or radiation was extracted. Chemotherapy information is not available through SEER. Overall survival was calculated using the Kaplan-Meier method. A multivariate analysis was performed to determine independent prognostic factors predicting for survival using a Cox proportional hazards model. Results: Fifty-eight percent of patients identified were male. The median age range at diagnosis was 65-69 years (range, 10-14 years – greater than 85 years). The most common histologic subtype in the present series was diffuse large B-cell lymphoma (DLBCL), which accounted for 71% of all patients. The 5-year overall survival for the group was 47%. Multivariate analysis indicates that age >60 and a marital status of single were predictive of a decreased cause-specific and overall-survival specific survival (p<0.05). Radiation therapy, but not surgery, was predictive of an improved overall survival (p<0.05). Conclusions: PPL is a rare form of extra-nodal NHLs of the GI tract. Adjuvant radiation therapy for patients with a PPL should be strongly considered and a surgical treatment should be avoided if an early diagnosis is established. A prospective study evaluating this patient population will be difficult given the rarity PPL. We hope that this case series will provide a context in evaluating and treating patients with PPL. No significant financial relationships to disclose.

2014 ◽  
Vol 120 (2) ◽  
pp. 300-308 ◽  
Author(s):  
Adam M. Sonabend ◽  
Brad E. Zacharia ◽  
Hannah Goldstein ◽  
Samuel S. Bruce ◽  
Dawn Hershman ◽  
...  

Object Central nervous system (CNS) hemangiopericytomas are relatively uncommon and unique among CNS tumors as they can originate from or develop metastases outside of the CNS. Significant difference of opinion exists in the management of these lesions, as current treatment paradigms are based on limited clinical experience and single-institution series. Given these limitations and the absence of prospective clinical trials within the literature, nationwide registries have the potential to provide unique insight into the efficacy of various therapies. Methods The authors queried the Surveillance Epidemiology and End Results (SEER) database to investigate the clinical behavior and prognostic factors for hemangiopericytomas originating within the CNS during the years 2000–2009. The SEER survival data were adjusted for demographic factors including age, sex, and race. Univariate and multivariate analyses were performed to identify characteristics associated with overall survival. Results The authors identified 227 patients with a diagnosis of CNS hemangiopericytoma. The median length of follow-up was 34 months (interquartile range 11–63 months). Median survival was not reached, but the 5-year survival rate was 83%. Univariate analysis showed that age and radiation therapy were significantly associated with survival. Moreover, young age and supratentorial location were significantly associated with survival on multivariate analysis. Most importantly, multivariate analysis using the Cox proportional hazards model showed a statistically significant survival benefit for patients treated with gross-total resection (GTR) in combination with adjuvant radiation treatment (HR 0.31 [95% CI 0.01–0.95], p = 0.04), an effect not appreciated with GTR alone. Conclusions The authors describe the epidemiology of CNS hemangiopericytomas in a large, national cancer database, evaluating the effectiveness of various treatment paradigms used in clinical practice. In this study, an overall survival benefit was found when GTR was accomplished and combined with radiation therapy. This finding has not been appreciated in previous series of patients with CNS hemangiopericytoma and warrants future investigations into the role of upfront adjuvant radiation therapy.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii390-iii391
Author(s):  
Aaron Mochizuki ◽  
Anna Janss ◽  
Sonia Partap ◽  
Paul Fisher ◽  
Yimei Li ◽  
...  

Abstract INTRODUCTION Medulloblastoma is one of the most common malignant brain tumors in children. To date, the treatment of average-risk (non-metastatic, completely resected) medulloblastoma includes craniospinal radiation therapy and adjuvant chemotherapy. Modern treatment modalities and now risk stratification of subgroups have extended the survival of these patients, exposing the long-term morbidities associated with radiation therapy. METHODS We performed a single-arm, multi-institution study, seeking to reduce the late effects of treatment in patients with average-risk medulloblastoma prior to advances in molecular subgrouping. To do so, we reduced the dose of craniospinal irradiation by 25% to 18 gray with the goal of maintaining the therapeutic efficacy as described in CCG 9892 with maintenance chemotherapy. RESULTS 28 patients aged 3–30 years were enrolled across three institutions between April 2001 and December 2010. Median age at enrollment was 9 years with a median follow-up time of 11.7 years. The 3-year relapse-free (RFS) and overall survival (OS) were 78.6% (95% CI 58.4% to 89.8%) and 92.9% (95% CI 74.4% to 98.2%), respectively. The 5-year RFS and OS were 71.4% (95% CI 50.1% to 84.6%) and 85.7% (95% CI 66.3% to 94.4%), respectively. Toxicities were similar to those seen in other studies; there were no grade 5 toxicities. CONCLUSIONS Given the known neurocognitive adverse effects associated with cranial radiation therapy, studies to evaluate the feasibility of dose reduction are needed. In this study, we demonstrate that select patients with average-risk medulloblastoma may benefit from reduced craniospinal radiation dose of 18 gray without impacting relapse-free or overall survival.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4676-4676
Author(s):  
Michael E. Confer ◽  
Jonathan D. Tward ◽  
Sherrie L. Perkins ◽  
Glen M. Bowen ◽  
Robert J. Lee ◽  
...  

Abstract INTRODUCTION: Non-mycosis fungoides (MF) primary cutaneous lymphoma (PCL) is rare, and the more indolent forms seldom progress to fatal, systemic lymphoma. Nevertheless, frequent relapses are common. Although several therapies exist, no standard of care has been established for initial treatment. OBJECTIVES: To compare the role of radiotherapy to other initial treatment options and to evaluate clinicopathologic factors affecting overall, cause-specific, and relapse-free survival METHODS: Thirty-eight patients from 1985 to 2006 were retrospectively identified and reviewed with non-MF PCLs including: primary cutaneous anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicle-center lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg type, or primary cutaneous intravascular large B-cell lymphoma. Regression-free, cause-specific, and overall survival was estimated using the methods of Kaplan and Meier. Outcomes were compared with the log-rank test and Cox regression analysis. RESULTS: 38 patients were included in the analysis with a median follow-up time of 34.6 months (range 2 – 138 months). The distribution of initial treatment was: surgery - 29%, topical therapy - 16%, systemic therapy - 18%, and radiation - 63%. Three patients never received radiation. For the entire cohort, the 5-year overall (OS), cause-specific (CSS), and relapse free survival (RFS) was 86.2%, 88.9%, and 29.5% respectively. Subjects who received radiation therapy (n=24) as part of their initial treatment course had a significantly longer median time to first relapse of 57 months compared to 3.2 months for the 14 subjects who did not receive radiotherapy (log-rank p &lt; 0.0001). Overall survival was significantly improved for subjects whose International Prognostic Index (IPI) score was 0–1 (n=25) versus those whose score was 2 or greater (n=13, p=0.05). Multivariate analysis for RFS revealed that the absence of radiation as part of initial treatment (Hazard Ratio (HR) = 22.2, 95% CI 2.1 – 238.5, p=0.01) and aggregate size less than 10cm (HR 0.04, 95% CI 0.0 – 0.3, p&lt;0.01) significantly altered the risk of relapse. No relapses were observed within the radiation therapy treatment field in 31/35 (89%) subjects following their first course of radiation therapy. Of the 15/35 (43%) of patients that relapsed anywhere following radiation, only 2/15 (13%) relapsed in-field exclusively, 2/15 (13%) relapsed both in and out-of-field, and the remaining 11/15 (73%) relapsed exclusively outside the area treated. No patient relapsed within the treatment field of after 24 months. CONCLUSION: An initial course of radiation therapy significantly delays relapse compared to other therapies for non-MF PCL and provides excellent local control of plaques. Our findings also extend the IPI as prognostic for overall survival for this rare disease. Bulky lesions greater than 10cm in any one dimension are more strongly correlated with relapse.


2019 ◽  
Vol 37 (7_suppl) ◽  
pp. 9-9
Author(s):  
Zachary D. Horne ◽  
Ryan P Smith ◽  
Sushil Beriwal ◽  
Ronny Kalash ◽  
Ashwin Shinde ◽  
...  

9 Background: Small cell prostate cancer (SCPC) is a rare entity with treatment patterns extrapolated from small cell cancer of the lung. Outcomes have been evaluated in small series but prognostic factors are relatively poorly defined. Methods: We utilized the National Cancer Data Base to analyze men diagnosed with SCPC from 2004-2015. Only men with known clinical TNM staging, treatment modalities, and follow up were included. Overall survival (OS) was analyzed and compared with Kaplan-Meier, log-rank, and Cox proportional hazards ratios. Associations with baseline and tumor properties were performed with Chi-squared, independent t-test, and bivariate regression analyses. Results: 800 men with SCPC were identified. Median PSA was 79.0 ng/dL. 55.6% of men had cM1 disease at diagnosis, 31.4% had cN0M0 disease, and 13.0% were cN1M0. Median follow up was 12.4 months for all patients and 19.3 months for cM0 patients. Median survival for cM1, cN0M0, and cN1M0 patients was 9.8, 28.5, and 17.1 months, respectively (p<0.001). In cM0 patients, 66 (18.7%) underwent radical prostatectomy (RP), 177 (50.1%) received radiation therapy (XRT), and 195 (45.2%) received chemotherapy (CT). Median survival for men undergoing RP was not reached vs those who did not undergo RP (p<0.001). XRT also showed a trend towards improved median OS (25.2 vs. 19.1 months, p=0.139). On multivariable analysis for cM0 men, only age (HR 1.044 [95% CI 1.025-10.64] p<0.001), cN1 (HR 1.378 [95% CI 1.001-1.898] p=0.050, RP (HR 0.429 [95% CI 0.259-0.709] p=0.001), and XRT (HR 0.520 [95% CI 0.384-0.704] p<0.001) were predictive for overall survival. When examining only men who received systemic therapy, XRT was the only additional treatment modality to exhibit a survival benefit (HR 0.623 [95% CI 0.425-0.912] p=0.015). Of men with cM1 disease, 78 (17.5%) underwent definitive local therapy (RP/XRT), but no difference in OS was observed. Conclusions: Small cell prostate cancer is an aggressive disease with the majority of men presenting with metastases. In those with pelvis-confined disease who are fit for systemic therapy, radiation therapy to the primary should be considered.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4584-4584
Author(s):  
Ahmed A Adel ◽  
Aimilia Exarchakou ◽  
Anas Hamad ◽  
Ruba Yasin ◽  
Hafedh Ghazouani ◽  
...  

Abstract Background: Lymphoma: either most common non-Hodgkin (NHL) or less common Hodgkin (HL), are well-known hematological malignancies. With advancement in treatment modalities, the survival in both lymphomas especially the "poor prognosis" non-Hodgkin lymphoma has evolved in the last decades. Hence, patient's outcome may be diverse and quite complicated; with some need extended time for observation, and others having multiple chemotherapy treatments. In this review, we will focus on the clinic-epidemiological patterns of various malignant lymphoma subtypes in Qatar in recent years (2013-2017) Objective: The primary aim is to investigate and compare the overall survival (OS) for both types of lymphoma; HL and NHL at 1, 3 and 5-years of follow up in adult lymphoma patients in Qatar between January 2013 - December 2017. Other objectives include comparing between the most frequent histological varieties, clinical and epidemiological characteristics of HL and NHL lymphoma in Qatar. The secondary objectives included clinical characteristics, treatments used, treatment response, disease-free survival and overall survival. Methods: A retrospective, descriptive study of consecutive cases was carried out at NCCCR, Qatar between 2013-2017. Inclusion criteria included: ≥ 18 years of age, male or female, any clinical stage at diagnosis, who had received any chemotherapy regimen, with a known outcome. Descriptive statistics was performed for all variables, and survival was assessed using Kaplan-Meier curves. Data was abstracted by Qatar National Cancer Registry and the 2008 World Health Organization (WHO) classification of hematopoietic and lymphoid tumors is used as reference for disease staging and pathological classification. We used STATA version 13.0 (StataCorp., College Station, TX) for exploratory data analysis and descriptive statistics. Results: During the period 2013-2017, 414 men and women were diagnosed with lymphoma in the state of Qatar. The median age at diagnosis being 49 years (interquartile range IQR 36-95 years; p&lt;0.001)) for all lymphoma patients combined. Males were more likely to develop both lymphoma types; HL and NHL than females; accounting for 2/3 of cases in each, yet statistically insignificant (74% and 70%, p=0.45). Based on subtypes, mature B-cell neoplasms (61 cases, 60%) were the most common among 13 identifiable NHL-B subtypes. Majority of HL cases belonged to Lymphocyte rich subtype (54 cases, 49%). With a median follow up of 17.3 months, the 1-year, 3-year and 5-year OS for the entire population of lymphoma patients were 99%, 82% and 64% (Figure 12). When stratified by major subtypes; HL and NHL, some trends became evident at 3-years follow-up (94% versus 82%). The 5-year OS were 67% and 60%, respectively. Throughout the study period, the OS in HL group were higher than NHL (p&lt;0.001), yet median OS was not reached. Conclusions: Diffuse large B-cell lymphoma constitutes the most frequent subtype for all lymphomas in Qatar. Overall, the survival was generally better for HL than NHL 67% and 60% respectively. Survival can be slightly deflated than other countries or regions especially HL, this is in part due to higher immigration rate in the country, so changes in survival over time (especially for longer periods) need to be examined alongside trends in incidence rates to interpret improvement in cancer control policies implemented. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 10065-10065
Author(s):  
M. Rivoire ◽  
S. Bonvalot ◽  
M. Castaing ◽  
E. Stoeckle ◽  
A. Laplanche ◽  
...  

10065 Background: A national retrospective study was conducted by the French Association of Surgery on primary RPS in order to define the best type of surgery and determine prognostic factors for abdominal recurrence and patient survival. There is still no consensus concerning the precise value of the extent of surgery and additional treatment modalities. Methods: Types of surgery were classified as: “systematic complete resection”, which is a systematic or partial resection of non-involved contiguous organs to achieve wide margins, “contiguously involved organ resection” and “simple complete resection”, a resection of the tumor with grossly negative margins. Survival was determined with the Kaplan-Meier method. Univariate and multivariate prognostic analyses were performed using logrank test and Cox model. Results: Data of 382 pts operated between 1985 and 2005 were retrospectively reviewed. Median age was 57 years (range 14–87). Median tumor size was 18 cm. Systematic complete resection, contiguously involved organ resection and simple complete resection were performed for 134 (35%), 153 (40%) and 87 (23%) pts, respectively. Eight pts (2%) had a biopsy without resection and 45 pts (12%) had macroscopic residual disease. Tumor was spilled during surgery for 76 pts (20%). Systematic complete resection and no spilling of the tumor were highly correlated with the number of pts operated by center (p-values <10-4). Histologic negative margins (136 pts, 36%) were highly correlated with systematic complete resection (p-value <10-4). Chemotherapy and radiotherapy were given to 135 (35%) and 122 pts (32%). Median survival was 6 years whereas median time to abdominal recurrence and metastasis were 4 and 15 years, respectively. On multivariate analysis, pre op biopsy, low grade, no spilling of the tumor, negative margins and number of pts operated by center were the significant factors predicting abdominal recurrence control whereas low grade, no spilling of the tumor and no macroscopic residual disease significantly predicted better overall survival. Conclusions: Tumor grade and quality of surgery were the two main prognostic factors for abdominal recurrence and overall survival. Surgical experience of these rare tumors and systematic complete resection decreased abdominal recurrence rate. No significant financial relationships to disclose.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4698-4698
Author(s):  
Grzegorz S. Nowakowski ◽  
Prabhjot Kaur ◽  
William R. Macon ◽  
Thomas M. Habermann ◽  
Kay Ristow ◽  
...  

Abstract Background: Primary Mediastinal Large B-cell Lymphoma (PMLBCL) is a biologically and pathologically distinct subset of large B-cell lymphoma of thymic origin. The natural history of PMLBCL is not well defined. While earlier reports suggested that PMLBCL is an aggressive disease with a poorer prognosis when compared to other large cell B cell lymphomas, recent studies report more favorable results. Some of the outcome variability reported in previous studies may be related to the inclusion of other pathological lymphoma subtypes involving mediastinum. Thus, we reviewed the pathology and clinical course of patients with PMLBCL seen at our institution. Methods: We reviewed the biopsies on all patients diagnosed with mediastinal B-cell non-Hodgkin lymphoma (NHL) between 10/1983 and 10/2004. The pathological criteria for diagnosis of PMLBCL was a diffuse infiltrate of neoplastic CD20+ large B-cells within the mediastinum that frequently had "clear" cytoplasm and were often associated with fibrosis. We then reviewed the clinical data for patients with a pathological diagnosis of PMLBCL. Results: 121 patients were diagnosed with mediastinal B cell NHL and had tissue available for review. Of these, 88 fulfilled the pathological diagnostic criteria for PMLBCL. The median age at presentation was 37 years (range 18–83). The male to female ratio was 1.06. Age adjusted IPI was: 0, 1, 2, 3 in 18%, 56%, 18% and 8% of the patients respectively. The most commonly used treatments were: CHOP (70%), R-CHOP (22%) and ProMACE-CYTABOM (6%). 62% of the patients received adjuvant radiotherapy. 24% of the patients received high dose chemotherapy with autologous stem cell transplantation (2 patients in first remission). The median follow up was 66 months. The 2 and 5 year survival was 72% and 68% respectively. On univariate analysis, age >60 (p=0.012) and presence of B symptoms (p=0.0094) were associated with an adverse outcome. On multivariate analysis, only the presence of B symptoms (p=0.039) was an independent predictor of shortened overall survival. The 2 year survival of patients with age adjusted IPI of 0–1 or 2–3 was 86% and 68% respectively (p=0.023). Patients treated with adjuvant radiation therapy had a 5 year survival of 74% versus 53% in patients who did not receive adjuvant radiation (p=0.042). However, there was a higher percentage of Ann Arbor stage 3 and 4 disease patients found in the group who did not receive radiotherapy treatment (66% vs 17% respectively). Conclusions: The overall survival rates seen in patients with a confirmed pathological diagnosis of PMLBCL were superior to that reported in earlier studies and similar to that reported with the use of more intensive chemotherapy regimens. While many authors report a high proportion of female patients with PMLBCL, we did not observe any gender differences. While patients receiving adjuvant radiation therapy had a better outcome than those that did not, the retrospective nature of this study and differences in stage distribution preclude conclusions regarding the role of adjuvant radiation therapy. Randomized studies will be necessary to assess the optimal treatment for PMLBCL.


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