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Öğe THE ESSENTIAL ROLE OF RADIOTHERAPY IN THE TREATMENT OF MERKEL CELL CARCINOMA: A STUDY FROM THE RARE CANCER NETWORK(Elsevier Science Inc, 2011) Ghadjar, Pirus; Kaanders, Johannes H.; Poortmans, Philipp; Zaucha, Renata; Krengli, Marco; Lagrange, Jean L.; Ozsoy, Orhan; Nguyen, Tan D.; Miralbell, Raymond; Baize, Adele; Boujelbene, Noureddine; Collen, Timothy; Scandolaro, Luciano; Untereiner, Michel; Goldberg, Hadassah; Pesce, Gianfranco A.; Anacak, Yavuz; Friedrich, Esther E.; Aebersold, Daniel M.; Beer, Karl T.Purpose: To evaluate the role of postoperative radiotherapy (RT) in Merkel cell carcinoma (MCC). Methods and Materials: A retrospective multicenter study was performed in 180 patients with MCC treated between February 1988 and September 2009. Patients who had had surgery alone were compared with patients who received surgery and postoperative RT or radical RT. Local relapse-free survival (LRFS), regional relapse-free survival (RRFS), and distant metastasis-free survival (DMFS) rates were assessed together with disease-free survival (DFS), cancer-specific survival (CSS), and overall survival (OS) rates. Results: Seventy-nine patients were male and 101 patients were female, and the median age was 73 years old (range, 38-93 years). The majority of patients had localized disease (n = 146), and the remaining patients had regional lymph node metastasis (n = 34). Forty-nine patients underwent surgery for the primary tumor without postoperative RT to the primary site; the other 131 patients received surgery for the primary tumor, followed by postoperative RT (n = 118) or a biopsy of the primary tumor followed by radical RT (n = 13). Median follow-up was 5 years (range, 0.2-16.5 years). Patients in the RT group had improved LRFS (93% vs. 64%; p < 0.001), RRFS (76% vs. 27%; p < 0.001), DMFS (70% vs. 42%; p = 0.01), DFS (59% vs. 4%; p < 0.001), and CSS (65% vs. 49%; p = 0.03) rates compared to patients who underwent surgery for the primary tumor alone; LRFS, RRFS, DMFS, and DFS rates remained significant with multivariable Cox regression analysis. However OS was not significantly improved by postoperative RT (56% vs. 46%; p = 0.2). Conclusions: After multivariable analysis, postoperative RT was associated with improved outcome and seems to be an important component in the multimodality treatment of MCC. (C) 2011 Elsevier Inc.Öğe Paraganglioma of the head and neck region, treated with radiation therapy, a Rare Cancer Network study(Wiley, 2019) Lassen-Ramshad, Yasmin; Ozyar, Enis; Alanyali, Senem; Poortmans, Philip; van Houtte, Paul; Sohawon, Schoeb; Esassolak, Mustafa; Krengli, Marco; Villa, Salvador; Miller, Robert; Demiroz, Candan; Akyurek, Serap; Aggerholm-Pedersen, Ninna; Thariat, JulietteBackground Paraganglioma of the head and neck (HNPGL) are rare often benign tumors. Surgery and radiation therapy (RT) are the main treatment choices. We present an analysis of outcome and toxicity after RT from 13 institutions of the Rare Cancer Network. Methods Data were collected using a questionnaire concerning patients' characteristics, treatment, and outcome. A total of 81 patients with 82 HNPGL were analyzed. Results The median follow-up was 48 months (1-456). Sixty-two lesions were treated with conventional RT and 20 lesions with stereotactic RT. Local control (LC) was achieved in 69 out of 77 lesions. Late toxicity occurred in 17 patients. Patients treated with stereotactic RT experienced neither disease progression nor late toxicity. Four patients with a follow-up longer than 20 years experienced disease progression. Conclusion RT for HNPGL offered good local control with acceptable toxicity. Stereotactic RT might offer better results. Long-term follow-up is required.Öğe The present and future opportunities of the Rare Cancer Network: an international consortium for advancement of oncologic care(Sage Publications Ltd, 2015) Sio, Terence T.; Mirimanoff, Rene-Olivier; Ozyar, Enis; Belkacemi, Yazid; Miller, Robert C.; Villa, Salvador; Thariat, Juliette; Krengli, Marco; Scandolaro, Luciano; Atalar, Banu; Ugurluer, Gamze; Gutierrez Garcia, Beatriz; Ashman, Jonathan B.; Anacak, Yavuz; Onal, Cem; Arat, Mutlu; Sun, Xu Shan; Tesanovic, Dusanka; Lassen-Ramshad, Yasmin; Oksuz, Didem; Dincbas, Fazilet; Sezen, Duygu; Akyurek, Serap; Kutuk, Tugce; Bolukbasi, Yasemin; Eren, Gulnihan; Paryani, Nitesh N.; Ahmed, Safia K.; Moretti, Luigi; Merrell, Kenneth W.; Chang, Kenneth; Mayeda, Mark; Arnett, Andrea L.; Habboush, Jacob Y.; Ozsahin, MahmutTo date, the Rare Cancer Network (RCN) has initiated more than 90 studies and 54 peer-reviewed publications were produced as a result. The Second International Symposium of the Rare Cancer Network recently took place in Istanbul, Turkey on April 17-18, 2015, and update was given on multiple currently ongoing projects, while also giving room for new proposals which will shape the direction of future studies for the group. This companion issue of the RCN Proceedings summarized the findings of this meeting, while also serving as a call for fresh projects and papers which will continue to energize the group and advance the oncologic science. A brief introduction to the principles, history, and vision of the RCN was also included. To review, the academic year of 2014-15 marked an enormous success for the international members of the RCN, with the generation of 8 fully published papers and more than 12 newly proposed topics. By the collective efforts of all RCN members, in the future, we look forward to the upcoming opportunities in continuing to advance the standard of chemo-and radiotherapeutic oncologic care for selected rare tumor topics. The studies of these rare cancers often do not allow the design and execution of prospectively enrolled trials; however, these uncommon malignancies do impact the humankind and add to its suffering globally in significant ways.Öğe Primary extranodal lymphoma of the glands. Literature review and options for best practice in 2019(Elsevier Science Inc, 2019) Belkacemi, Yazid; Sio, Terence T.; Colson-Durand, Laurianne; Onal, Cem; Villa, Salvador; Anacak, Yavuz; Krengli, Marco; Thariat, Juliette; Ugurluer, Gamze; Miller, Robert C.; Mirimanoff, Rene-Olivier; Ozsahin, Mahmut; Nhu Hanh ToPrimary extranodal non-Hodgkin's lymphomas (EN-NHL) are a heterogeneous group of malignancies that involve numerous entities with significant difference in terms of tumor site locations, prognostic factors, biology expression, and therapeutic options. In the literature, many EN-NHL types were reported from limited series which only allowed narrow views for elucidating prognostic factors and defining the role of loco-regional therapies in the era of new systemic and biologically targeted therapies. The Rare Cancer Network (RCN), an international multidisciplinary consortium, has published a number of reports on several EN-NHL sites which included many gland locations. In this review, we will focus on the recent literature for a selected number of EN-NHL types in both exocrine and endocrine gland locations. We aim to provide renewed and clear messages for the best practice in 2019 for diagnosis, histopathology, treatments, and also their prognostic implications. We believe that better understanding of molecular and genetic characteristics of these particular diseases is crucial for an appropriate management in the era of personalized treatment developments.Öğe Reply to "Proper tumor classification and growth rate are key elements when considering indications and results of radiotherapy for head and neck paragangliomas"(Wiley, 2019) Lassen-Ramshad, Yasmin; Ozyar, Enis; Alanyali, Senem; Poortmans, Philip; Van Houtte, Paul; Sohawon, Schoeb; Esassolak, Mustafa; Krengli, Marco; Villa, Salvador; Miller, Robert C.; Demiroz, Candan; Akyurek, Serap; Aggerholm-Pedersen, Ninna; Thariat, Juliette
