The fate of paratesticular masses: 13 years’ experience in a tertiary referral centre

Abstract Objective Paratesticular neoplasms exhibit different behaviours, depending on the embryological tissue of origin. Treatment modalities can depend on the differential diagnosis. The aim of this study is to present the clinical, morphological and histopathological features of patients with paratesticular masses and their follow-ups and is intended to increase awareness of the issues. Methodology We included 31 excisions of paratesticular masses, after radiological diagnosis as paratesticular mass in our hospital between 2007–2020. Information on treatment modalities, tumour recurrence, metastasis, and survival rates were obtained from hospital archives. All patients were evaluated by taking patients’ history, physical examination, scrotal ultrasound, chest radiography, and serum tumour markers. Treatment modality was selected according to intraoperative findings. Haematoxylin-eosin sections were examined, and immunohistochemical analyses were performed for smooth muscle actin, desmin, Ki67, CD34, S100, and myogenin. Ten high-power fields were counted to document Ki67 and p53 nuclear positivity rates. Results A total of 31 operations were performed with recurrence in three patients. Histomorphological and immunohistochemical examination revealed eleven malignant masses; eight rhabdomyosarcomas, a leiomyosarcoma, a liposarcoma and a large B cell lymphoma. Other excised masses were benign and infective lesions. Conclusion Paratesticular masses are heterogeneous tumours that follow different clinical courses. Clinicians must be aware of this histological diversity in order to plan a treatment pathway. This study is one of the largest published series, with a long follow-up period. It shows that the most critical features in determining prognosis are histopathological subtype and tumour grade.

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Trends in Survival of Patients with Primary Gastric Diffuse Large B-Cell Lymphoma: An Analysis of 7051 Cases in the SEER Database

Disease Markers ◽

10.1155/2018/7473935 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7

Author(s):

Pan-pan Liu ◽

Yi Xia ◽

Xi-wen Bi ◽

Yu Wang ◽

Peng Sun ◽

...

Keyword(s):

B Cell ◽

Cell Lymphoma ◽

Survival Rates ◽

Multivariable Analysis ◽

B Cell Lymphoma ◽

Treatment Modalities ◽

Seer Database ◽

Limited Information ◽

Large B Cell Lymphoma ◽

Large B Cell

Treatment modalities for primary gastric diffuse large B-cell lymphoma (PG-DLBCL) have changed significantly during the past decades. However, limited information on the trends of clinical outcome of PG-DLBCL patients has been reported. Here, we conducted a retrospective analysis using the Surveillance, Epidemiology, and End Results (SEER) database to compare the survival trends of PG-DLBCL patients from 1973 to 2014. Patients were divided into 2 eras based on the year of diagnosis in relation to immunotherapy with the anti-CD20 antibody rituximab that was approved in 1997 and became a widely used drug in 2000. There was a significant improvement in survival among PG-DLBCL patients diagnosed in the 2001–2014 era (n=4186) compared to patients diagnosed in the 1973–2000 era (n=2865), with the 5-year overall survival rates of 53% and 47%, respectively (p=0.001). Multivariable analysis revealed that the 2001–2014 era (HR = 0.892, p=0.001) was associated with lower mortality and that patients of older age, Black race, advanced stage, and male gender were associated with poor prognosis. Although outcome of PG-DLBCL has significantly improved over time, more effective therapies are needed for older patients to further improve their survival.

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Epidemiologic and Clinical Patterns of Lymphoma in Qatar 2013-2017: A Population-Based Cohort Study

Blood ◽

10.1182/blood-2021-144637 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 4584-4584

Author(s):

Ahmed A Adel ◽

Aimilia Exarchakou ◽

Anas Hamad ◽

Ruba Yasin ◽

Hafedh Ghazouani ◽

...

Keyword(s):

Overall Survival ◽

B Cell ◽

Conflicts Of Interest ◽

Disease Free Survival ◽

B Cell Lymphoma ◽

Descriptive Statistics ◽

World Health ◽

Treatment Modalities ◽

Epidemiological Characteristics

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<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<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.

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Comparison of adjuvant chemoradiation to perioperative chemotherapy for the treatment of resected gastric and gastroesophageal junction adenocarincoma.

Journal of Clinical Oncology ◽

10.1200/jco.2015.33.3_suppl.139 ◽

2015 ◽

Vol 33 (3_suppl) ◽

pp. 139-139

Author(s):

Howard John Lim ◽

Hae Rin Kim ◽

Devin Schellenberg ◽

Christian K. Kollmannsberger ◽

Winson Y. Cheung

Keyword(s):

Gastroesophageal Junction ◽

Survival Rates ◽

Patient Characteristics ◽

Operative Management ◽

Descriptive Statistics ◽

Treatment Modalities ◽

Pharmacy Records ◽

Adjuvant Chemoradiation ◽

Kaplan Meier

139 Background: There are several accepted peri-operative treatment modalities for resected gastric (GC) and gastroesophageal junction (GEJ) adenocarcinoma. In 2008, peri-operative chemotherapy (CT) using the MAGIC protocol was adopted as the preferred approach over adjuvant chemoradiation with the MacDonald protocol (cXRT) in British Columbia. An era to era comparison was performed to determine if there were differences in outcomes. Methods: Data from pharmacy records of patients (pts) referred to 1 of 5 cancer treatment centres in BC were analyzed from Jan 2001-July 2010. Pts that underwent curative resection for GC or GEJ were included. The cXRT cohort was defined as those treated from Jan 2001-Dec 2007, prior to the introduction of CT. The CT cohort included those treated from Jan 2008-July 2010. Descriptive statistics were used to compare the groups. Survival analysis was performed using Kaplan Meier methods. Results: Table 1 summarizes the patient characteristics. In the CT arm, there were more males, fewer pts with a LN ratio >0.2, and shorter median follow-up. 92.1% completed pre-operative chemotherapy and 44.7% completed post-operative chemotherapy whereas 73.3% of pts completed cXRT (p<0.05). Median survival was 37.5 and 36.9 months in the CT and cXRT arms, respectively. Conclusions: Delivery of CT was consistent with the MAGIC trial whereas more patients completed cXRT than in the MacDonald trial (73.3% vs. 64%). Outcomes of CT compared to cXRT appear to be similar in this comparative analysis with similar relapse and survival rates. Pre-operative CT results in fewer pts with a LN ratio > 0.2. Either modality can be considered in the peri-operative management of GC or GEJ adenocarcinoma. [Table: see text]

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Clinical Outcome of Patients with Follicular Lymphoma and Bulky Disease After Rituximab-CHOP Immunochemotherapy with and without Consolidating Radiotherapy.

Blood ◽

10.1182/blood.v114.22.2722.2722 ◽

2009 ◽

Vol 114 (22) ◽

pp. 2722-2722

Author(s):

Fabienne McClanahan ◽

Thomas Hielscher ◽

Michael Rieger ◽

Manfred Hensel ◽

Kai Neben ◽

...

Keyword(s):

Follicular Lymphoma ◽

Conflicts Of Interest ◽

Survival Rates ◽

B Cell Lymphoma ◽

Bulky Disease ◽

Mediastinal Lymphoma ◽

Significant Difference ◽

Tumour Bulk ◽

New Location

Abstract Abstract 2722 Poster Board II-698 Background: As the clinical management of patients with bulky disease remains challenging, many centres apply involved-field radiotherapy (IF-RT) after completion of immunochemotherapy. This strategy remains controversial. Patients and Methods: To evaluate the benefit of consolidating IF-RT in addition to immunochemotherapy, we retrospectively analyzed relapse patterns and survival of patients presenting with bulky follicular lymphoma (FL). Bulky disease was defined as abdominal/ mediastinal lymphoma mass >7.5 cm and/ or peripheral lymphoma mass >5 cm. All patients were treated within a prospective randomized trial on 126 patients with FL with six cycles of standard CHOP-chemotherapy in combination with 1, 3 or 6 cycles of Rituximab, followed by consolidating IF-RT in patients with bulky disease. 42 eligible patients with bulky disease were identified and form the foundation of this analysis, of which 26 were irradiated and 16 were not, violating the protocol. Results: With the exception of number of affected nodal regions, there was no significant difference between the irradiated and the non-irradiated group with regards to presenting characteristics (p > .05). Among all patients, bulks were located below the diaphragm in 91%. A second tumour bulk was present in 9 patients (22%). Female to male ratio was 1.3:1, and the median age at diagnosis was 54 years (range 23–73). According to FLIPI, 10% were classified as low risk (0–1), 45% as intermediate risk (2), and 45% as high risk (3–5). B symptoms were absent in 69%. Eleven patients (26 %) had received one course of Rituximab, 17 (41%) three courses and 14 (33%) six courses. There was no significant difference between the irradiated and the non-irradiated group with regards to previous exposure to immunochemotherapy (p = .628). After a median follow-up of 60 months, a total of 21 patients (50%) had progressed or relapsed and 9 patients (21%) had died. With the exception of one patient who died from congestive heart failure following chemotherapy-related cardiomyopathy, the main cause of death was disease progression or relapse. Highly malignant transformation into diffuse large B-cell lymphoma was observed in 5 cases. In the irradiated group, relapse occurred in 12 of 26 patients. Half of these relapses were located within the original bulk or within the bulk plus a new location, and 50% at a new location altogether; 42% occurred within the previously irradiated area. In the non-irradiated group, 9 of 16 patients relapsed. The corresponding rates regarding relapse location were 67% for original plus new location and 33% for new location only. There was no statistically significant difference between exposure to radiotherapy after immunochemotherapy and the likelihood of a relapse per se (p = .751) or at a specific location (p = .66). At the last follow-up, 31% of irradiated patients had achieved CR and 23% PR. Among the non-irradiated group, the corresponding rates were 25% and 19%. There was no significant difference in remission rates at any staging examination throughout the clinical trial between the two treatment groups (p > .05). 6-year progression-free- and overall survival rates were 52% and 80% after IF-RT and 48% and 73% without IF-RT (p = 1.00, p = .68 respectively). Conclusion: In this analysis, there was no difference in relapse rate, relapse location, PFS and OS between patients with bulky FL treated with and without consolidating IF-RT. Although patient numbers are limited, this is the first analysis of its kind conducted in the Rituximab era. Disclosures: No relevant conflicts of interest to declare.

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Subglottic haemangioma in children: experience with open surgical excision

The Journal of Laryngology & Otology ◽

10.1017/s0022215106003586 ◽

2006 ◽

Vol 120 (12) ◽

pp. 1033-1037 ◽

Cited By ~ 18

Author(s):

Y Bajaj ◽

B E J Hartley ◽

M E Wyatt ◽

D M Albert ◽

C M Bailey

Keyword(s):

Surgical Excision ◽

Treatment Modalities ◽

Direct Result ◽

Tertiary Referral Centre ◽

Threatening Condition ◽

Life Threatening ◽

One Stop ◽

Open Excision ◽

Subglottic Haemangioma

Subglottic haemangioma is a potentially life-threatening condition for which various treatment modalities are available. The objective of this study was to evaluate our results for open excision of subglottic haemangioma. The study assessed 18 patients who had been treated at a paediatric tertiary referral centre. Most of these patients (83.3 per cent) had undergone open surgical excision without post-operative tracheostomy and had been intubated for several days post-operatively (single-stage procedure). In most of these patients (66.7 per cent), an anterior cartilage graft had been used for reconstruction. The average follow up in this study was 25 months. All the patients in this series had achieved an adequate airway after the procedure. One patient had developed a recurrence of haemangioma in the trachea at a later date. The results of open surgical excision in this study were very encouraging. Seventeen out of 18 (94.4 per cent) patients had avoided tracheostomy or had been decannulated as a direct result of surgery. One of these 18 patients (5.6 per cent) had required a temporary post-operative tracheostomy for 13 months as the subglottis cleared; this was classed as a partial success. Our experience is that open excision is a highly successful ‘one stop’ treatment for subglottic haemangioma, which avoids prolonged use of steroids and multiple endoscopic procedures. No patient in this series developed subglottic stenosis, which can be a significant complication of laser application.

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Estimation of Long-Term Survival with Tafasitamab + Lenalidomide in Relapsed/Refractory Diffuse Large B-Cell Lymphoma

Blood ◽

10.1182/blood-2020-140398 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 9-10

Author(s):

Gilles Salles ◽

Budhaditya Goswami ◽

Vincenzo Bagnardi ◽

Debarshi Dey ◽

Mark Winderlich ◽

...

Keyword(s):

Weibull Distribution ◽

Survival Benefit ◽

Survival Rates ◽

B Cell Lymphoma ◽

Parametric Models ◽

Cure Model ◽

Mixture Cure Model ◽

Advisory Role

Background L-MIND (NCT02399085) is an ongoing, open-label, single-arm, Phase II study of tafasitamab + lenalidomide (LEN) in patients with relapsed/refractory (R/R) diffuse large B-cell lymphoma (DLBCL) who are ineligible for autologous stem cell transplant. Progression-free survival (PFS) estimates the treatment effect of the study drug only, while overall survival (OS) also includes effects of potential subsequent cancer treatments or palliative care. Given that not all OS and PFS events have occurred after a long follow-up duration, traditional survival analyses may underestimate survival benefit for tafasitamab + LEN by assuming the same mortality rate for the whole population. We used mixture cure models to estimate the proportion of long-term survivors (LTS) and survival benefit associated with tafasitamab + LEN treatment. Methods In the L-MIND study (data cut-off: Nov 30, 2019), 80/81 enrolled patients with R/R DLBCL received tafasitamab + LEN, with a median and maximum follow-up duration of 31.8 months (95% CI: 27.2-35.9) and 43.5 months, respectively. The Kaplan-Meier (KM) plots of PFS and OS were observed to plateau after 30 months, with the majority of events reported up to that time-point and very few events beyond this time-point. Patients without a PFS event or patients who did not decease after the observed plateau exhibit a high survival benefit from tafasitamab + LEN treatment and are considered LTS. Thus, the patient population can be considered to consist of two sub-populations - LTS and non-LTS. A "mixture cure model" was fitted on the PFS and OS data to estimate the proportion of LTS and associated mean survival for the two sub-populations (Lambert PC, et al. 2007). To incorporate background mortality (Bansal, et al. 2019), age- and sex-specific US mortality rates (US CDC 2017) were factored in for the entire study population (enrolled in Europe and US). The weighted average of the mean survival for the two sub-populations provided an estimate of the mean survival for the whole population. For comparative purposes, standard parametric models without considering an LTS proportion were fitted on PFS and OS data additionally. Sensitivity analyses were performed considering different survival distributions (exponential distribution, Weibull distribution and log-logistic distribution). Results By using a mixture cure model fitted to PFS and OS data separately, the estimated proportion of LTS for the tafasitamab + LEN combination was 42% (95% CI: 30-55) and 47% (95% CI: 29-66), respectively. The predicted mean survival when mixture cure model was fitted on PFS data was 16.7 years for LTS patients vs 0.5 years for non-LTS patients, yielding 7.3 years for the overall population. Under this mixture cure model, the survival rates of patients with tafasitamab + LEN treatment were 40.2% and 36.0% at 2 and 5 years. Similar results were obtained when the mixture cure model was fitted on OS data. Using a standard parametric model (Weibull distribution), the predicted mean survival in the overall population was 2.8 years fitting PFS data (Figure 1) and 4.5 years fitting OS data (Figure 2). Conclusions PFS and OS KM curves for the L-MIND study reaching a long plateau and not following a Weibull distribution suggest the presence of an LTS subgroup. Standard parametric models may lead to a biased estimation of the OS benefit in such situations. The mixture cure model suggests that the treatment of R/R DLBCL patients with tafasitamab + LEN is associated with a high LTS proportion. The survival rates of patients with tafasitamab + LEN treatment were 40.2% and 36.0% at 2 and 5 years. Disclosures Salles: Takeda:Honoraria;BMS/Celgene:Honoraria, Other: consultancy or advisory role;Debiopharm:Consultancy, Honoraria, Other: consultancy or advisory role;Genmab:Honoraria, Other;Karyopharm:Honoraria;Kite, a Gilead Company:Honoraria, Other: consultancy or advisory role ;Epizyme:Honoraria, Other: consultancy or advisory role;Janssen:Honoraria, Other: consultancy or advisory role;Abbvie:Other: consultancy or advisory role;Roche:Honoraria, Other: consultancy or advisory role;Novartis:Honoraria, Other: consultancy or advisory role;MorphoSys:Honoraria, Other: consultancy or advisory role;Autolos:Other: consultancy or advisory role.Goswami:MorphoSys AG:Ended employment in the past 24 months.Bagnardi:MorphoSys AG:Consultancy.Dey:MorphoSys AG:Current Employment.Winderlich:MorphoSys AG:Current Employment.Ambarkhane:MorphoSys AG:Current Employment.Huang:MorphoSys AG:Current Employment.Nowakowski:Curis:Consultancy;Kymera:Consultancy;Kite:Consultancy;Ryvu:Consultancy, Membership on an entity's Board of Directors or advisory committees;Celgene/BMS:Consultancy, Research Funding;Seattle Genetics:Consultancy;MorphoSys:Consultancy, Research Funding;NanoString:Research Funding.

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Primary Intracranial Malignant Melanomas: Retrospective Analysis of Management in a Single Chinese Institution and literature Review

10.21203/rs.3.rs-927214/v1 ◽

2021 ◽

Author(s):

Lifeng Chen ◽

Yang Yang ◽

Dongmei Li ◽

Bo Bu ◽

xiaodong ma

Keyword(s):

Overall Survival ◽

Preoperative Diagnosis ◽

Treatment Options ◽

Survival Rates ◽

Radical Resection ◽

Treatment Modalities ◽

Subtotal Resection ◽

Mri Features ◽

Diagnosis Rate

Abstract Background: Primary intracranial malignant melanoma (PIMM) is a rare malignant tumor. The authors retrospectively reviewed and discussed the clinical features, treatment modalities, and clinical outcomes of patients with histologically proven PIMM. Methods: The data of 15 patients with PIMM in our hospital within 14 years (from January 2005 to January 2019) were collected. The clinical and imaging presentations, pathology, surgical strategies, adjuvant treatment and the prognosis were analyzed in this study.Results: Eleven men and 4 women with mean age 37.9 years (19-61 years) were observed over an average follow-up period of 22.6 months (range, 6–36 months). CT showed iso or high density in 12 cases (80%). MRI sacns indicated that 14 tumors were mainly hyperintensity on T1 weighted images, hypointensity on T2 weighted images, and had no or mild enhancement. The treatment modalities included total resection followed by conventional radiotherapy (RT) (n=12), and subtotal resection followed by stereotactic radiosurgery (SRS) (n=3). Fifteen cases had recurrence or metastasis at the average 14.7 months (6-23 months): local recurrence (8 cases), distant metastasis (5 cases), both of them (2 cases). Fourteen cases (93.3%) died and the mean overall survival was 22 months (6-36 months). The median survival period was 23 months. The overall survival rates at 1, 2 and 3 years were 80%, 47%, and 13%, respectively. Radical resection with RT was associated with longer overall survival (log-rank, p<0.05). Conclusions: PIMM is an extremely rare tumor with poor prognosis, which is difficult to get correct preoperative diagnosis. Improvement of the recognition of MRI features of melanoma can increase the preoperative diagnosis rate, and radical resection with RT may provide longer overall survival rate. Targeted and immunotherapy therapies may provide promise as treatment options for PIMM.

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Head and Neck Cancer a Single Institution Experience: King Abdulaziz University

Journal of King Abdulaziz University-Medical Sciences ◽

10.4197/med.22-3.2 ◽

2015 ◽

Vol 22 (3) ◽

pp. 9-17

Author(s):

Rolina K Al-Wassia ◽

Nisreen A. Awad ◽

Shadi S. Al-Khayyat ◽

Atlal M. Abusanad ◽

Hani Z. Al-Marzouki ◽

...

Keyword(s):

Head And Neck Cancer ◽

Head And Neck ◽

Neck Cancer ◽

Survival Rates ◽

Treatment Modalities ◽

Clinical Practices ◽

Hospital Data ◽

Intensity Modulated ◽

Regional Control

The purpose of this study is to assess the loco-regional control and overall survival in head and neck cancer patients, as well as evaluate the clinical benefit of intensity-modulated radiotherapy implemented in 2011 at our Hospital. Data of 117 patients between 2007 and 2014 was reviewed retrospectively. Cumulative survival and disease control rates were calculated by Kaplan-Meier product-limit actuarial method. Loco-regional control and survival rates for intensity modulated and three-dimensional conformal radiotherapy were compared by a logistic regression test. After a median follow-up of 12 months, 53 (51%) patients who underwent radical radiotherapy were free of disease, 43 (42%) with disease, and seven (7%) unknown. During this time, 31 (26%) patients died from the disease. Using actuarial estimates for the two-year follow-up, this study found that significant gains in survival were obtained by switching treatment modalities. The benchmarking gives reassurance that our results are comparable to the best clinical practices internationally.

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Natural History of C282Y Homozygotes for Hemochromatosis

Canadian Journal of Gastroenterology ◽

10.1155/2002/161569 ◽

2002 ◽

Vol 16 (5) ◽

pp. 297-302 ◽

Cited By ~ 43

Author(s):

John P Wojcik ◽

Mark R Speechley ◽

Ann E Kertesz ◽

Subrata Chakrabarti ◽

Paul C Adams

Keyword(s):

Clinical Symptoms ◽

Survival Rates ◽

Tertiary Referral Centre ◽

Actuarial Survival ◽

Term Survival ◽

Long Term Survival ◽

Hemochromatosis Gene ◽

Screening Study

PURPOSE: To study the clinical outcomes of subjects who are homozygous for the C282Y mutation of the hemochromatosis gene.SUBJECTS AND METHODS: All patients referred to a tertiary referral centre for hemochromatosis were included. The study also included 16 C282Y homozygotes detected in a population screening study.RESULTS: The study comprised 277 C282Y homozygotes, including 16 nonexpressing C282Y homozygotes. The mean follow-up period was 7.3 years (range zero to 44 years). The actuarial survival rates of C282Y homozygotes at five, 10 and 20 years were 95%, 93% and 66%, respectively. Life-threatening diseases (cirrhosis, hepatocellular carcinoma, diabetes, heart disease) were present in 36% of male C282Y homozygotes and 19% of female C282Y homozygotes. Cirrhosis of the liver and diabetes were the major clinical symptoms affecting long term survival. Only one nonexpressing homozygote required venesection therapy during the follow-up period.CONCLUSIONS: Long term survival is excellent in C282Y homozygotes diagnosed and treated before the development of cirrhosis and diabetes.

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Impact of Rituximab and Methotrexate on the Survival of the Patients with Sinonasal Tract Diffuse Large B-Cell Lymphoma

Blood ◽

10.1182/blood.v128.22.1859.1859 ◽

2016 ◽

Vol 128 (22) ◽

pp. 1859-1859 ◽

Cited By ~ 1

Author(s):

Pauli Vähämurto ◽

Susanna Mannisto ◽

Marjukka Pollari ◽

Marja-Liisa Karjalainen-Lindsberg ◽

Antti A. Mäkitie ◽

...

Keyword(s):

Research Funding ◽

Survival Rates ◽

B Cell Lymphoma ◽

Patient Characteristics ◽

Curative Intent ◽

Large B Cell Lymphoma ◽

Kaplan Meier ◽

Risk Of Progression ◽

Group 5

Abstract Background: Diffuse large B-cell lymphoma (DLBCL) of the sinonasal track (SNT) is a rare presentation of the aggressive lymphoid malignancy with the incidence of 0.06-0.17 per 100 000 in the Western population. SNT involvement is associated with increased risk of central nervous system (CNS) progression, and thus eligible patients are often treated with chemoimmunotherapy in combination with CNS prophylaxis with intrathecally (it) or intravenously (iv) administered methotrexate (MTX). However, the data demonstrating that the addition of CD20 antibody rituximab and/or CNS penetrating MTX improves the outcome of the patients with SNT-DLBCL are lacking. Materials and Methods: The aim of the study was to characterize the clinical findings of the patients with SNT-DLBCL treated at two University Hospital districts in Finland (Helsinki and Tampere University Hospitals). The hospital records of 59 patients were retrospectively reviewed on parameters for patient demographics, tumor characteristics, treatment and outcome. Results: Forty-five patients were treated with curative intent with CHOP-like chemotherapy, 24 (53%) of them with chemoimmunotherapy containing rituximab (R+) and 21 (47%) before the rituximab era (R-). Among the patients treated with curative intent, iv MTX was given to 24 patients (53%; M+ group), whereas the remaining 21 patients (47%) did not receive MTX (M- group). The median age was 65 years for the whole cohort, and 64 years for the patients treated with curative intent. The patients treated with curative intent had better performance status in comparison to all patients. Otherwise, the patient characteristics were similar. Follow-up data was collected to 60 months. Median follow-up time for the entire cohort was 47 months. No differences were observed in the patient characteristics between the R+ and R- groups. The patients in the M+ group were younger than the patients in the M- group (67% vs 24% were <60 years, p=0.007). Otherwise, no significant differences in the patient characteristics were found between the two groups. MTX was used equally often during the pre-R and R eras. The patients in the R+ group had lower risk of progression (RR 0.384, 95% CI 0.145-1.018, p=0.054) and death (RR 0.235, 95% CI 0.066-0.836, p=0.025) in comparison to the patients in the R- group. According to Kaplan-Meier analyses, the patients in the R+ group had better survival rates than the patients in the R- group (5-y progression free survival (PFS) 66% vs 38%, p=0.046; 5-y overall survival (OS) 80% vs 43%, p=0.015). Addition of MTX to chemotherapy also reduced the risk of progression (RR 0.384, 95% CI 0.151-0.977, p=0.044) and death (RR 0.253, 95% CI 0.080-0.795, p=0.019). According to Kaplan-Meier analyses, the patients in the M+ group had better survival rates than the patients in the M- group (5-y PFS, 67% vs 31%, p=0.036; 5-y OS 82% vs 35%, p=0.011). Only one patient in the cohort experienced CNS progression. Conclusions: SNT-DLBCL patients treated with curative intent with R containing regimen have superior survival in comparison to the patients treated in the pre-R era. Likewise, intravenously administered CNS penetrating MTX improves survival. In this cohort, only one patient experienced CNS progression, and thus the impact of different treatments on the risk of CNS progression could not be evaluated. Disclosures Mannisto: SOBI: Honoraria; Pfizer: Honoraria; Gilead: Other: Travel expenses; Celgene: Other: Travel expenses; Novartis: Other: Travel expenses; Amgen: Other: Travel expenses; Takeda: Honoraria, Other: Travel expenses; Roche: Honoraria, Other: Travel expenses. Leppä:Amgen: Research Funding; Takeda: Honoraria, Other: Travel expenses; Bayer: Honoraria, Research Funding; Mundipharma: Research Funding; Roche: Honoraria, Other: Travel Expenses, Research Funding; Janssen: Research Funding; CTI Life Sciences: Honoraria.

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