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Philip Poortmans - One of the best experts on this subject based on the ideXlab platform.

  • management of adenoid cystic carcinoma of the breast a rare Cancer Network study
    International Journal of Radiation Oncology Biology Physics, 2012
    Co-Authors: K Khanfir, Yazid Belkacemi, Adel Kallel, Sylviane Villette, Claire Vautravers, Tan Dat Nguyen, Robert C Miller, Alphonse G Taghian, L Boersma, Philip Poortmans
    Abstract:

    Background: Mammary adenoid cystic carcinoma (ACC) is a rare breast Cancer. The aim of this retrospective study was to assess prognostic factors and patterns of failure, as well as the role of radiation therapy (RT), in ACC. Methods: Between January 1980 and December 2007, 61 women with breast ACC were treated at participating centers of the Rare Cancer Network. Surgery consisted of lumpectomy in 41 patients and mastectomy in 20 patients. There were 51(84%) stage pN0 and 10 stage cN0 (16%) patients. Postoperative RT was administered to 40 patients (35 after lumpectomy, 5 after mastectomy). Results: With a median follow-up of 79 months (range, 6–285), 5-year overall and disease-free survival rates were 94% (95% confidence interval [CI], 88%–100%) and 82% (95% CI, 71%–93%), respectively. The 5-year locoregional control (LRC) rate was 95% (95% CI, 89%–100%). Axillary lymph node dissection or sentinel node biopsy was performed in 84% of cases. All patients had stage pN0 disease. In univariate analysis, survival was not influenced by the type of surgery or the use of postoperative RT. The 5-year LRC rate was 100% in the mastectomy group versus 93% (95% CI, 83%–100%) in the breast-conserving surgery group, respectively (p = 0.16). For the breast-conserving surgery group, the use of RT significantly correlated with LRC (p = 0.03); the 5-year LRC rates were 95% (95% CI, 86%–100%) for the RT group versus 83% (95% CI, 54%–100%) for the group receiving no RT. No local failures occurred in patients with positive margins, all of whom received postoperative RT. Conclusion: Breast-conserving surgery is the treatment of choice for patients with ACC breast Cancer. Axillary lymph node dissection or sentinel node biopsy might not be recommended. Postoperative RT should be proposed in the case of breast-conserving surgery. 2012 Elsevier Inc. Adenoid cystic carcinoma, Breast Cancer, Radiotherapy, Surgery.

  • primary breast lymphoma patient profile outcome and prognostic factors a multicentre rare Cancer Network study
    BMC Cancer, 2008
    Co-Authors: Wendy Jeanneretsozzi, Philip Poortmans, Sylviane Villette, Alphonse G Taghian, Ron Epelbaum, Daniel R Zwahlen, Beat Amsler, Y Belkacemi, Tan Nguyen, Pierre Scalliet
    Abstract:

    Background To asses the clinical profile, treatment outcome and prognostic factors in primary breast lymphoma (PBL).

  • prognostic factors in solitary plasmacytoma of the bone a multicenter rare Cancer Network study
    BMC Cancer, 2006
    Co-Authors: David Knobel, Philip Poortmans, Yazid Belkacemi, Christine Landmann, A Zouhair, Richard W Tsang, M Bolla, Fazilet Dincbas Oner, B Castelain, Mahmut Ozsahin
    Abstract:

    Background: Solitary plasmacytoma (SP) of the bone is a rare plasma-cell neoplasm. There are no conclusive data in the literature on the optimal radiation therapy (RT) dose in SP. Therefore, in this large retrospective study, we wanted to assess the outcome, prognostic factors, and the optimal RT dose in patients with SP. Methods: Data from 206 patients with bone SP without evidence of multiple myeloma (MM) were collected. Histopathological diagnosis was obtained for all patients. The majority (n = 169) of the patients received RT alone; 32 chemotherapy and RT, and 5 surgery. Median follow-up was 54 months (7–245). Results: Five-year overall survival, disease-free survival (DFS), and local control was 70%, 46%, and 88%; respectively. Median time to MM development was 21 months (2–135) with a 5-year probability of 51%. In multivariate analyses, favorable factors were younger age and tumor size < 5 cm for survival; younger age for DFS; anatomic localization (vertebra vs. other) for local control. Older age was the only predictor for MM. There was no dose-response relationship for doses 30 Gy or higher, even for larger tumors. Conclusion: Younger patients, especially those with vertebral localization have the best outcome when treated with moderate-dose RT. Progression to MM remains the main problem. Further investigation should focus on adjuvant chemotherapy and/or novel therapeutic agents.

  • management of primary anal canal adenocarcinoma a large retrospective study from the rare Cancer Network
    International Journal of Radiation Oncology Biology Physics, 2003
    Co-Authors: Yazid Belkacemi, Philip Poortmans, Marco Krengli, A Zouhair, Christine Berger, Gaelle Piel, Jeanbaptiste Meric, T D Nguyen, Franck Behrensmeier, Abdelkarim S Allal
    Abstract:

    Abstract Purpose Primary adenocarcinoma of the anus is a rare tumor. The current standard treatment consists of abdominoperineal resection (APR). The aim of this Rare Cancer Network study was to evaluate the prognostic factors and outcome after the three most commonly used treatment approaches. Methods and materials This multicenter study collected data from 82 patients: 15 with T1 (18%), 34 with T2 (42%), 22 with T3 (27%), and 11 with T4 (13%) tumors according to the TNM classification (International Union Against Cancer, 1997). Patients were separated into, and analyzed according to, three treatment categories: radiotherapy/surgery (RT/S group, n = 45), combined radiochemotherapy (RT/CHT group, n = 31), and APR alone (APR group, n = 6). The main patient characteristics were evenly distributed among the three groups. Results The actuarial locoregional relapse rate at 5 years was 37%, 36%, and 20%, respectively, in the RT/S, RT/CHT, and APR groups (RT/S vs. RT/CHT, p = 0.93; RT/CH vs. APR, p = 0.78). The 3-, 5-, and 10-year overall survival rate was 47%, 29%, and 23% in the RT/S group, 75%, 58%, and 39% in the RT/CHT group, and 42%, 21%, and 21% in the APR group (RT/CHT vs. RT/S, p = 0.027), respectively. The 5- and 10-year disease-free survival rate was 25% and 18% in the RT/S group, 54% and 20% in the RT/CHT group, and 22% and 22% in the APR group (RT/CHT vs. RT/S, p = 0.038), respectively. Multivariate analysis revealed four independent prognostic factors for survival: T stage, N stage, histologic grade, and treatment modality. Conclusion Primary adenocarcinoma of the anal canal requires rigorous management. Multivariate analysis showed that T and N stage, histologic grade, and treatment modality are independent prognostic factors for survival. We observed better survival rates after combined RT/CHT. We also recommend using APR only for salvage treatment.

  • outcome and prognostic factors in orbital lymphoma a rare Cancer Network study on 90 consecutive patients treated with radiotherapy
    International Journal of Radiation Oncology Biology Physics, 2003
    Co-Authors: Sylvie Martinet, Christoph Oehlere, L. Scandolaro, Philip Poortmans, Marco Krengli, Yazid Belkacemi, Philippe Maingon, Mahmut Ozsahin, Christine Landmann, Raymond Miralbell
    Abstract:

    Abstract Purpose: To assess the outcome and prognostic factors in patients with orbital lymphoma treated by radiotherapy (RT). Methods and Materials: Between 1980 and 1999, 90 consecutive patients with primary orbital lymphoma were treated in 13 member institutions of the Rare Cancer Network. A full staging workup was completed in 56 patients. Seventy-eight patients had low-, 6 intermediate-, and 6 high-grade lymphoma, and 75 had a single orbital localization. All patients underwent RT with a median dose of 34.2 Gy (range 4.0–50.4). Eleven patients received chemotherapy in addition to RT. Results: After RT, local control was achieved in 97% of the patients. Local progression occurred in 2% and local relapse 1%. The rate of systemic relapse was 20%, and 9% of the patients developed metachronous contralateral eye involvement. The 5-year disease-free survival, overall survival, and cause-specific survival rate was 65%, 78%, and 87%, respectively. In univariate analyses, the statistically significant favorable prognostic factors were younger age, low grade, normal erythrocyte sedimentation rate, absence of muscular infiltration, complete response to treatment, conjunctival localization, and normal lactate dehydrogenase value for overall survival, disease-free survival, and freedom from treatment failure. In multivariate analysis, the favorable factors were younger age and low grade for overall and disease-free survival; a favorable response, conjunctival localization, and complete staging were highly significant for disease-free survival and freedom from treatment failure. Neither the RT technique nor the total dose influenced the outcome. Cataract and xerophthalmia were the most prominent late toxicities. Conclusion: Moderate- to low-dose RT alone is able to control primary orbital lymphoma with low morbidity. A full staging workup is warranted in these patients. Prognostic factors were identified that could be useful in the overall management of this uncommon site of primary lymphoma.

Joyce C Niland - One of the best experts on this subject based on the ideXlab platform.

  • radiation for diffuse large b cell lymphoma in the rituximab era analysis of the national comprehensive Cancer Network lymphoma outcomes project
    Cancer, 2015
    Co-Authors: Bouthaina S Dabaja, Ann Vanderplas, Myron S Czuczman, Joyce C Niland, Leo I Gordon, Allison Crosbythompson, Gregory A Abel, Jonathan W Friedberg, Mark S Kaminski, Michael Millenson
    Abstract:

    BACKGROUND The role of consolidation radiotherapy was examined for patients with diffuse large B-cell lymphoma who were treated at institutions of the National Comprehensive Cancer Network during the rituximab era. METHODS Failure-free survival (FFS) and overall survival (OS) were analyzed in terms of patient and treatment characteristics. Potential associations were investigated with univariate and multivariate survival analysis and matched pair analysis. RESULTS There were 841 patients, and most (710 or 84%) received 6 to 8 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP); 293 (35%) received consolidation radiation therapy (RT). Failure occurred for 181 patients: 126 patients (70%) who did not receive RT and 55 patients (30%) who did. At 5 years, both OS and FFS rates were better for patients who had received RT versus those who did not (OS, 91% vs 83% [P = .01]; FFS, 83% vs 76% [P = .05]). A matched pair analysis (217 pairs matched by age, stage, International Prognostic Index [IPI] score, B symptoms, disease bulk, and response to chemotherapy) showed that the receipt of RT improved OS (hazard ratio [HR], 0.53 [P = .07]) and FFS (HR, 0.77 [P = .34]) for patients with stage III/IV disease, but too few events took place among those with stage I/II disease for meaningful comparisons (HR for OS, 0.94 [P = .89]; HR for FFS, 1.81 [P = .15]). A multivariate analysis suggested that the IPI score and the response to chemotherapy had the greatest influence on outcomes. CONCLUSIONS There was a trend of higher OS and FFS rates for patients who had received consolidation RT after R-CHOP (especially for patients with stage III/IV disease), but the difference did not reach statistical significance. Cancer 2014. © 2014 American Cancer Society. Cancer 2015;121:1032–1039. © 2014 American Cancer Society.

  • patterns of use of 18 fluoro 2 deoxy d glucose positron emission tomography for initial staging of grade 1 2 follicular lymphoma and its impact on initial treatment strategy in the national comprehensive Cancer Network non hodgkin lymphoma outcomes d
    Leukemia & Lymphoma, 2013
    Co-Authors: Karim Abounassar, Ann Vanderplas, Maria A Rodriguez, Allison L Crosby, Myron S Czuczman, Joyce C Niland, Gregory A Abel, Jonathan W Friedberg, Michael Millenson, Leo I Gordon
    Abstract:

    AbstractWe describe the patterns of use of 18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) for the initial staging of patients with newly diagnosed grade 1–2 follicular lymphoma (FL) and its potential impact on treatment. Data were obtained from the National Comprehensive Cancer Network Non-Hodgkin Lymphoma Outcomes database. Patients who presented between 1 January 2001 and 30 September 2009 with newly diagnosed grade 1–2 FL, with at least 6 months of follow-up, were included. We identified 953 eligible patients and 532 (56%) underwent FDG-PET as part of initial staging. Among patients who underwent FDG-PET for initial staging, 438 (82%) received early treatment compared to 259 (61.5%) of those staged without FDG-PET (p < 0.0001). Of all patients with stage I FL (n = 100), 47% were treated with radiotherapy (RT) alone, and the choice of initial treatment strategy for stage I FL did not vary significantly by use of FDG-PET (p = 0.22). The use of FDG-PET for staging of FL is widespread a...

  • time to adjuvant chemotherapy for breast Cancer in national comprehensive Cancer Network institutions
    Journal of the National Cancer Institute, 2013
    Co-Authors: J L Vandergrift, Joyce C Niland, Richard L Theriault, Stephen B Edge, Yuning Wong, Loretta Loftus, Tara M Breslin, Clifford A Hudis, Sara H Javid, Hope S Rugo
    Abstract:

    A number of clinical trials demonstrating the benefit of adjuvant chemotherapy have been published over the past 20 years (1) and clinical practice guidelines recommend chemotherapy for many breast Cancer patients following completion of definitive surgery to reduce the risk of recurrence (2). The optimal time interval between diagnosis and initiation of adjuvant chemotherapy is unclear. Long intervals between surgery and chemotherapy have been associated with poorer disease-specific outcomes (3–5), although null associations between time to chemotherapy (TTC) and outcomes have also been reported (6). No studies were identified that examined the impact of the diagnosis to chemotherapy interval on patient outcomes. Currently, several professional societies endorse time-dependent quality measures. For example, one of the American Society of Clinical Oncology (ASCO)/National Comprehensive Cancer Network (NCCN) quality measures recommends adjuvant chemotherapy within 120 days of diagnosis for women aged less than 70 years with stage II or stage III hormone receptor–negative breast Cancer (7). In reviewing concordance with this measure in NCCN centers, Hughes et al. (8) found that treatment for 87% of patients met the quality measure; however, 6% of patients (47% of nonconcordant patients) were nonconcordant because chemotherapy began more than 120 days after diagnosis. In this analysis, we sought to examine the sociodemographic, clinical, and treatment factors associated with an increased TTC initiation at NCCN centers. Our goals were to characterize patients who might be at increased risk for delay and to identify potentially mutable factors contributing to delay.

  • postoperative adjuvant chemotherapy use in patients with stage ii iii rectal Cancer treated with neoadjuvant therapy a national comprehensive Cancer Network analysis
    Journal of Clinical Oncology, 2013
    Co-Authors: Polina Khrizman, Joyce C Niland, Anna Ter Veer, Dana Milne, Kelli Bullard Dunn, William E Carson, Paul F Engstrom, Stephen Shibata, John M Skibber, Martin R Weiser
    Abstract:

    Purpose Practice guidelines recommend that patients who receive neoadjuvant chemotherapy and radiation for locally advanced rectal Cancer complete postoperative adjuvant systemic chemotherapy, irrespective of tumor downstaging. Patients and Methods The National Comprehensive Cancer Network (NCCN) Colorectal Cancer Database tracks longitudinal care for patients treated at eight specialty Cancer centers across the United States and was used to evaluate how frequently patients with rectal Cancer who were treated with neoadjuvant chemotherapy also received postoperative systemic chemotherapy. Patient and tumor characteristics were examined in a multivariable logistic regression model. Results Between September 2005 and December 2010, 2,073 patients with stage II/III rectal Cancer were enrolled in the database. Of these, 1,193 patients receiving neoadjuvant chemoradiotherapy were in the analysis, including 203 patients not receiving any adjuvant chemotherapy. For those seen by a medical oncologist, the most fr...

  • clinicopathologic features patterns of recurrence and survival among women with triple negative breast Cancer in the national comprehensive Cancer Network
    Cancer, 2012
    Co-Authors: Nan Lin, Ann Vanderplas, Joyce C Niland, Richard L Theriault, Melissa E Hughes, Stephen B Edge, Yuning Wong, Douglas W Blayney, Eric P Winer
    Abstract:

    BACKGROUND: The objective of this study was to describe clinicopathologic features, patterns of recurrence, and survival according to breast Cancer subtype with a focus on triple-negative tumors. METHODS: In total, 15,204 women were evaluated who presented to National Comprehensive Cancer Network centers with stage I through III breast Cancer between January 2000 and December 2006. Tumors were classified as positive for estrogen receptor (ER) and/or progesterone receptor (PR) (hormone receptor [HR]-positive) and negative for human epidermal growth factor receptor 2 (HER2); positive for HER2 and any ER or PR status (HER2-positive); or negative for ER, PR, and HER2 (triple-negative). RESULTS: Subtype distribution was triple-negative in 17% of women (n = 2569), HER2-positive in 17% of women (n = 2602), and HR-positive/HER2-negative in 66% of women (n = 10,033). The triple-negative subtype was more frequent in African Americans compared with Caucasians (adjusted odds ratio, 1.98; P < .0001). Premenopausal women, but not postmenopausal women, with high body mass index had an increased likelihood of having the triple-negative subtype (P = .02). Women with triple-negative Cancers were less likely to present on the basis of an abnormal screening mammogram (29% vs 48%; P < .0001) and were more likely to present with higher tumor classification, but they were less likely to have lymph node involvement. Relative to HR-positive/HER2-negative tumors, triple-negative tumors were associated with a greater risk of brain or lung metastases; and women with triple-negative tumors had worse breast Cancer-specific and overall survival, even after adjusting for age, disease stage, race, tumor grade, and receipt of adjuvant chemotherapy (overall survival: adjusted hazard ratio, 2.72; 95% confidence interval, 2.39-3.10; P < .0001). The difference in the risk of death by subtype was most dramatic within the first 2 years after diagnosis (overall survival for 0-2 years: OR, 6.10; 95% confidence interval, 4.81-7.74). CONCLUSIONS: Triple-negative tumors were associated with unique risk factors and worse outcomes compared with HR-positive/HER2-negative tumors. Cancer 2012. © 2012 American Cancer Society.

Jeanpierre Jeannon - One of the best experts on this subject based on the ideXlab platform.

David Cella - One of the best experts on this subject based on the ideXlab platform.

  • content validity of the national comprehensive Cancer Network functional assessment of Cancer therapy breast Cancer symptom index nfbsi 16 and patient reported outcomes measurement information system promis physical function short form with advanced
    Health and Quality of Life Outcomes, 2019
    Co-Authors: Meaghan Krohe, Derek H Tang, Brittany Klooster, Dennis A Revicki, Nina Galipeau, David Cella
    Abstract:

    The purpose of this study is to evaluate the content validity of the National Comprehensive Cancer Network – Functional Assessment of Cancer Therapy – Breast Cancer Symptom Index (NFBSI-16) and the Patient-Reported Outcomes Measurement Information System (PROMIS) Physical Function Short Form 10b among patients with hormone receptor positive (HR+)/human epidermal growth factor receptor 2 negative (HER2-) advanced breast Cancer. Cognitive debriefing interviews sought to evaluate patients’ ability to read, understand, and meaningfully respond to the questionnaires, as well as to evaluate the questionnaires’ relevance in the target patient population. Interviews were conducted by telephone and lasted approximately 90 min. Audio recordings were transcribed, anonymized, and analyzed using qualitative data analysis software. Fifteen cognitive debriefing interviews were conducted with women (mean age 66.0 years [standard deviation = 12.4]). Patients reported metastases in the bone (86.7%), liver (20.0%), lung (13.3%), skin (6.7%), and lymph node (6.7%) (not mutually exclusive). All patients for whom data were available demonstrated understanding of the instructions and the recall period of the NFBSI-16 (n = 14/14, 100.0%) and the PROMIS (n = 14/14, 100.0%). Greater than 90% of patients demonstrated understanding of each of the items in the NFBSI-16 and the PROMIS. Greater than 70% of patients demonstrated understanding of the response options of the NFBSI-16, > 90% understood response options of PROMIS Items 1–6, and ≥ 50% understood response options of PROMIS Items 7–10. Conceptual relevance was supported for most items in both questionnaires based on patients’ reports of experiencing the concepts as part of their breast Cancer experience. The results of the cognitive debriefing interviews provide evidence that the NFBSI-16 and PROMIS Physical Function Short Form 10b have content validity in the HR+/HER2- advanced breast Cancer patient population. Patients may benefit from additional instructions at the point the response options reverse direction in the PROMIS.

  • development and validation of a symptom index for advanced hepatobiliary and pancreatic Cancers the national comprehensive Cancer Network functional assessment of Cancer therapy nccn fact hepatobiliary pancreatic symptom index nfhsi
    Cancer, 2012
    Co-Authors: Zeeshan Butt, Sarah Rosenbloom, Jennifer L Beaumont, Neehar D Parikh, Karen L Syrjala, Amy P Abernethy, Al B Benson, David Cella
    Abstract:

    BACKGROUND: The 45-item Functional Assessment of Cancer Therapy-Hepatobiliary (FACT-Hep) questionnaire assesses health-related quality of life in patients with liver, bile duct, and pancreatic Cancers. Although the FACT-Hep was initially derived from patient input, this study's researchers sought to verify adequate coverage of items by soliciting open-ended input from patients with advanced disease. METHODS: As part of a larger study in collaboration with the National Comprehensive Cancer Network (NCCN), 50 people (60% male, 80% caucasian, average age 60.4 years) with stage 3 or 4 hepatobiliary or pancreatic Cancer were recruited. Participants generated and ranked up to 10 important symptoms and concerns that physicians should monitor when assessing the value of chemotherapy. Patients were also able to provide open-ended, qualitative information that was evaluated systematically. Ten expert physicians also provided input on priority symptoms. RESULTS: The resulting 18-item NCCN-FACT Hepatobiliary-Pancreatic Symptom Index (NFHSI-18) demonstrated high internal consistency (α = .89) and moderate to strong correlations with measures of physical well-being (ρ = .76), emotional well-being (ρ = 0.52), and functional well-being (ρ = 0.57). Scores on the NFHSI-18 were also highly correlated with the original hepatobiliary scale of the FACT-Hep (ρ = .82; all P < .001). Compared with patients with better performance status, patients with poor performance status had worse NFHSI-18 symptom scores, F(3,47) = 9.74; P = .0003. CONCLUSIONS: The NFHSI-18 assesses symptoms of importance to patients with hepatobiliary and pancreatic Cancers and demonstrates promising measurement properties. The scale is a good candidate for brief symptom assessment in clinical trials.

  • priority symptoms in advanced breast Cancer development and initial validation of the national comprehensive Cancer Network functional assessment of Cancer therapy breast Cancer symptom index nfbsi 16
    Value in Health, 2012
    Co-Authors: Sofia F Garcia, Sarah Rosenbloom, Jennifer L Beaumont, Douglas E Merkel, Jamie H Von Roenn, Deepa Rao, David Cella
    Abstract:

    Abstract Objectives By using methods consistent with recent regulatory guidance on patient-reported outcomes as endpoints in clinical trials, we created a new version of the Functional Assessment of Cancer Therapy-Breast Cancer Symptom Index (FBSI), with emphasis on patient input during the development process. Methods We obtained input on the most important symptoms to monitor during treatment for stage III or IV breast Cancer from 52 patients recruited from National Comprehensive Cancer Network institutions as well as support service organizations. Participating patients shared their top-priority symptoms/concerns through open-ended interviews and symptom checklists. To ensure adequate content coverage, we evaluated results alongside the original version of the FBSI, which was created on the basis of a survey of oncology clinicians at National Comprehensive Cancer Network institutions and items in the Functional Assessment of Chronic Illness Therapy measurement system. We also obtained input from 10 National Comprehensive Cancer Network oncologists regarding whether symptoms were primarily related to disease or treatment. Results We selected breast Cancer–related symptoms and concerns endorsed as high priority by both oncology patients and clinicians for inclusion in the new National Comprehensive Cancer Network-Functional Assessment of Cancer Therapy-Breast Cancer Symptom Index-16 (NFBSI-16), which includes all eight items from the original FBSI and eight additional items from Functional Assessment of Chronic Illness Therapy measures. The NFBSI-16 is formatted by subscale: Disease-Related Symptom, Treatment Side-Effect, and General Function and Well-Being. Results provide preliminary support for NFBSI-16's internal consistency reliability ( α = 0.87) and validity as evidenced by moderate-to-strong relationships with expected criteria. Conclusions Reflecting the priority symptoms of breast Cancer patients and clinicians, the NFBSI-16 can be used to help evaluate the effectiveness of treatments for advanced breast Cancer in clinical practice and research.

Marco Krengli - One of the best experts on this subject based on the ideXlab platform.

  • radiotherapy in the treatment of extracranial hemangiopericytoma solitary fibrous tumor study from the rare Cancer Network
    Radiotherapy and Oncology, 2020
    Co-Authors: Marco Krengli, Tiziana Cena, Thomas Zilli, B A Jereczekfossa, Berardino De Bari, Salvador Villa Freixa, Johannes H A M Kaanders, S Torrente, D Pasquier
    Abstract:

    Abstract Background and purpose The role of radiotherapy (RT) in the treatment of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) is still under debate. We aimed at investigating whether radiotherapy can improve the results in patients operated for extracranial HPC/SFT. Materials and methods Data from patients with HPC/SFT, treated from 1982 to 2012, were retrospectively reviewed within the Rare Cancer Network framework. Actuarial local control (LC), disease-free survival (DFS), metastasis-free survival (MFS) and overall survival (OS) were calculated with Kaplan-Meyer method. Patient and tumor parameters were analyzed by univariate and multivariate analysis. Results Of 114 HPC/SFT, 58 (50.9%) occurred in the extremities/superficial trunk and 56 (49.1%) in intra-thoracic/retroperitoneum. Seventy-eight patients (68.4%) underwent surgery only (Sx), and 36 (31.6%) Sx and RT (Sx + RT). Median RT dose was 60 Gy (range 45–68.4 Gy) in 1.6–2.2 Gy fractions. In the extremities/superficial trunk group of patients, actuarial 5-year LC rates were 50.4% after Sx and 91.6% after Sx + RT (p  Conclusion Addition of RT to Sx could improve the prognosis, in terms of LC and DFS, essentially in patients with extremities/superficial trunk tumor locations.

  • atypical and malignant meningioma outcome and prognostic factors in 119 irradiated patients a multicenter retrospective study of the rare Cancer Network
    International Journal of Radiation Oncology Biology Physics, 2007
    Co-Authors: D Pasquier, Marco Krengli, Stefan Bijmolt, Theo Veninga, Nicolas Rezvoy, S Villa, Damien C Weber, Brigitta G Baumert, Emine Canyilmaz, Deniz Yalman
    Abstract:

    PURPOSE: To retrospectively analyze and assess the outcomes and prognostic factors in a large number of patients with atypical and malignant meningiomas. METHODS AND MATERIALS: Ten academic medical centers participating in this Rare Cancer Network contributed 119 cases of patients with atypical or malignant meningiomas treated with external beam radiotherapy (EBRT) after surgery or for recurrence. Eligibility criteria were histologically proven atypical or anaplastic (malignant) meningioma (World Health Organization Grade 2 and 3) treated with fractionated EBRT after initial resection or for recurrence, and age >18 years. Sex ratio (male/female) was 1.3, and mean (+/-SD) age was 57.6 +/- 12 years. Surgery was macroscopically complete (Simpson Grades 1-3) in 71% of patients; histology was atypical and malignant in 69% and 31%, respectively. Mean dose of EBRT was 54.6 +/- 5.1 Gy (range, 40-66 Gy). Median follow-up was 4.1 years. RESULTS: The 5- and 10-year actuarial overall survival rates were 65% and 51%, respectively, and were significantly influenced by age >60 years (p = 0.005), Karnofsky performance status (KPS) (p = 0.01), and high mitotic rate (p = 0.047) on univariate analysis. On multivariate analysis age >60 years (p = 0.001) and high mitotic rate (p = 0.02) remained significant adverse prognostic factors. The 5- and 10-year disease-free survival rates were 58% and 48%, respectively, and were significantly influenced by KPS (p = 0.04) and high mitotic rate (p = 0.003) on univariate analysis. On multivariate analysis only high mitotic rate (p = 0.003) remained a significant prognostic factor. CONCLUSIONS: In this multicenter retrospective study, age, KPS, and mitotic rate influenced outcome. Multicenter prospective studies are necessary to clarify the management and prognostic factors of such a rare disease.

  • management of primary anal canal adenocarcinoma a large retrospective study from the rare Cancer Network
    International Journal of Radiation Oncology Biology Physics, 2003
    Co-Authors: Yazid Belkacemi, Philip Poortmans, Marco Krengli, A Zouhair, Christine Berger, Gaelle Piel, Jeanbaptiste Meric, T D Nguyen, Franck Behrensmeier, Abdelkarim S Allal
    Abstract:

    Abstract Purpose Primary adenocarcinoma of the anus is a rare tumor. The current standard treatment consists of abdominoperineal resection (APR). The aim of this Rare Cancer Network study was to evaluate the prognostic factors and outcome after the three most commonly used treatment approaches. Methods and materials This multicenter study collected data from 82 patients: 15 with T1 (18%), 34 with T2 (42%), 22 with T3 (27%), and 11 with T4 (13%) tumors according to the TNM classification (International Union Against Cancer, 1997). Patients were separated into, and analyzed according to, three treatment categories: radiotherapy/surgery (RT/S group, n = 45), combined radiochemotherapy (RT/CHT group, n = 31), and APR alone (APR group, n = 6). The main patient characteristics were evenly distributed among the three groups. Results The actuarial locoregional relapse rate at 5 years was 37%, 36%, and 20%, respectively, in the RT/S, RT/CHT, and APR groups (RT/S vs. RT/CHT, p = 0.93; RT/CH vs. APR, p = 0.78). The 3-, 5-, and 10-year overall survival rate was 47%, 29%, and 23% in the RT/S group, 75%, 58%, and 39% in the RT/CHT group, and 42%, 21%, and 21% in the APR group (RT/CHT vs. RT/S, p = 0.027), respectively. The 5- and 10-year disease-free survival rate was 25% and 18% in the RT/S group, 54% and 20% in the RT/CHT group, and 22% and 22% in the APR group (RT/CHT vs. RT/S, p = 0.038), respectively. Multivariate analysis revealed four independent prognostic factors for survival: T stage, N stage, histologic grade, and treatment modality. Conclusion Primary adenocarcinoma of the anal canal requires rigorous management. Multivariate analysis showed that T and N stage, histologic grade, and treatment modality are independent prognostic factors for survival. We observed better survival rates after combined RT/CHT. We also recommend using APR only for salvage treatment.

  • outcome and prognostic factors in orbital lymphoma a rare Cancer Network study on 90 consecutive patients treated with radiotherapy
    International Journal of Radiation Oncology Biology Physics, 2003
    Co-Authors: Sylvie Martinet, Christoph Oehlere, L. Scandolaro, Philip Poortmans, Marco Krengli, Yazid Belkacemi, Philippe Maingon, Mahmut Ozsahin, Christine Landmann, Raymond Miralbell
    Abstract:

    Abstract Purpose: To assess the outcome and prognostic factors in patients with orbital lymphoma treated by radiotherapy (RT). Methods and Materials: Between 1980 and 1999, 90 consecutive patients with primary orbital lymphoma were treated in 13 member institutions of the Rare Cancer Network. A full staging workup was completed in 56 patients. Seventy-eight patients had low-, 6 intermediate-, and 6 high-grade lymphoma, and 75 had a single orbital localization. All patients underwent RT with a median dose of 34.2 Gy (range 4.0–50.4). Eleven patients received chemotherapy in addition to RT. Results: After RT, local control was achieved in 97% of the patients. Local progression occurred in 2% and local relapse 1%. The rate of systemic relapse was 20%, and 9% of the patients developed metachronous contralateral eye involvement. The 5-year disease-free survival, overall survival, and cause-specific survival rate was 65%, 78%, and 87%, respectively. In univariate analyses, the statistically significant favorable prognostic factors were younger age, low grade, normal erythrocyte sedimentation rate, absence of muscular infiltration, complete response to treatment, conjunctival localization, and normal lactate dehydrogenase value for overall survival, disease-free survival, and freedom from treatment failure. In multivariate analysis, the favorable factors were younger age and low grade for overall and disease-free survival; a favorable response, conjunctival localization, and complete staging were highly significant for disease-free survival and freedom from treatment failure. Neither the RT technique nor the total dose influenced the outcome. Cataract and xerophthalmia were the most prominent late toxicities. Conclusion: Moderate- to low-dose RT alone is able to control primary orbital lymphoma with low morbidity. A full staging workup is warranted in these patients. Prognostic factors were identified that could be useful in the overall management of this uncommon site of primary lymphoma.