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Item The present and future opportunities of the Rare Cancer Network: an international consortium for advancement of oncologic care(SAGE PUBLICATIONS LTD, 2015-01-01) 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, Mahmut; Network, Rare CancTo 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 trialsItem Primary hepatic lymphoma: a retrospective, multicenter Rare Cancer Network study(PAGEPRESS PUBL, 2016-01-01) Ugurluer, Gamze; Miller, Robert C.; Li, Yexiong; Thariat, Juliette; Ghadjar, Pirus; Schick, Ulrike; Ozsahin, MahmutPrimary hepatic lymphoma (PHL) is a rare malignancy. We aimed to assess the clinical profile. outcome and prognostic factors in PHL through the Rare Cancer Network (RCN). A retrospective analysis of 41 patients was performed. Median age was 62 years (range. 2386 years) with a male-to-female ratio of 1.9:1.0. Abdominal pain or discomfort was the most common presenting symptom. Regarding B-symptoms, 19.5\% of patients had fever, 17.1\% weight loss, and 9.8\% night sweats. The most common radiological presentation was multiple lesions. Liver function tests were elevated in 56.1\% of patients. The most common histopathological diagnosis was diffuse large B-cell lymphoma (65.9\%). Most of the patients received Chop-like (cyclophosphamide. doxorubicin, vincristine, and prednisone) regimens, 4 patients received radiotherapy (dose range, 30.6-40.0 Gy). Median survival was 163 months, and 5- and 10-year overall survival rates were 77 and 59\%, respectively. The 5- and 10-year disease-free and lymphoma-specific survival rates were 69, 56, 87 and 70\%, respectively. Multivariate analysis revealed that fever. weight loss, and normal hemoglobin level were the independent factors influencing the outcome. In this retrospective multicenter RCN study, patients with PHL had a relatively better prognosis than that reported elsewhere. Multicenter prospective studies are still warranted to establish treatment guidelines, outcome and prognostic factors.Item Primary extranodal lymphoma of the glands. Literature review and options for best practice in 2019(ELSEVIER SCIENCE INC, 2019-01-01) 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; To, Nhu HanhPrimary 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.Item The Rare Cancer Network: ongoing studies and future strategy(SAGE PUBLICATIONS LTD, 2014-01-01) Ozsahin, Mahmut; Mirimanoff, Rene-Olivier; Thariat, Juliette; Sun, Xu Shan; Atalar, Banu; Lassen-Ramshad, Yasmin; Ugurluer, Gamze; Krishnan, Sunil; Hallemeier, Christopher; Van Houtte, Paul; Krengli, Marco; Zhang, Lan Jun; Chang, Kenneth; Funk, Ryan; Rooney, Jessica; Miller, Robert C.The Rare Cancer Network (RCN) was formed in the early 1990's to create a global network that could pool knowledge and resources in the studies of rare malignancies whose infrequency prevented both their study with prospective clinical trials. To date, the RCN has initiated 74 studies resulting in 46 peer reviewed publications. The First International Symposium of the Rare Cancer Network took place in Nice in March of 2014. Status updates and proposals for new studies were heard for fifteen topics. Ongoing studies continue for cardiac sarcomas, thyroid cancers, glomus tumors, and adult medulloblastomas. New proposals were presented at the symposium for primary hepatic lymphoma, solitary fibrous tumors, Rosai-Dorfman disease, tumors of the ampulla of Vater, salivary gland tumors, anorectal melanoma, midline nuclear protein in testes carcinoma, pulmonary lymphoepithelioma-like carcinoma, adenoid cystic carcinoma of the trachea, osteosarcomas of the mandible, and extra-cranial hemangiopericytoma. This manuscript presents the abstracts of those proposals and updates on ongoing studies, as well a brief summary of the vision and future of the RCN.Item Neuroprotective effects of erythropoietin against oxidant injury following brain irradiation: an experimental study(TERMEDIA PUBLISHING HOUSE LTD, 2016-01-01) Ugurluer, Gamze; Cebi, Aysegul; Mert, Handan; Mert, Nihat; Serin, Meltem; Erkal, Haldun SukruIntroduction: Radiation therapy (RT) is a major treatment modality, and the central nervous system is a dose-limiting organ in clinical RT. This experimental study aims to present the evaluation of the neuroprotective effects of erythropoietin (EPO) against oxidant injury following brain irradiation in rats. Material and methods: Forty Wistar rats were randomly assigned to four groups (n = 10 each). In group 1 the rats received no EPO and underwent sham RT. The rats in groups 2 and 3 received EPO. In group 2 rats underwent sham RT, while in group 3 rats received RT. The rats in group 4 received no EPO and underwent RT. Rats were irradiated using a Cobalt-60 teletherapy machine using a single fraction of 20 Gy covering the whole brain. Cervical dislocation euthanasia was performed. The nitrite and malondialdehyde (MDA) levels and the superoxide dismutase (SOD) and glutathione peroxidase (GSHPX) activities were evaluated in dissected brain tissues. Results: The nitrite and MDA levels were higher in the RT group (2.10 +/-0.62 ppm, 26.02 +/-2.16 nmol/mlItem Primary pericardial extragastrointestinal stromal tumor: A case report and literature review(SPANDIDOS PUBL LTD, 2015-01-01) Arpaci, Taner; Tokat, Fatma; Arpaci, Rabia Bozdogan; Akbas, Tugana; Ugurluer, Gamze; Yavuz, SinanGastrointestinal stromal tumors (GISTs) are the most prevalent mesenchymal tumors of the gastrointestinal tract. GISTs are considered to originate from the interstitial cells of Cajal, the pacemakers of the peristaltic activity of the gastrointestinal tract. More than 95\% of GISTs express KIT protein and discovered on GIST-1. GISTs may also be encountered in locations outside the gastrointestinal tract, in which case they are referred to as extra-GISTs (EGISTs) and often behave more aggressively. This is the case report of a primary pericardial EGIST in a 53-year-old male patient, confirmed by immunohistochemistry. To the best of our knowledge, this is the third case of EGIST diagnosed above the diaphragm, without being associated with the esophageal wall. Two cases of primary EGIST arising from the pleura were reported previously. In addition, this is the first reported case of an EGIST originating from the pericardium.Item Multi-Institutional Outcomes of Stereotactic Magnetic Resonance Image Guided Adaptive Radiation Therapy With a Median Biologically Effective Dose of 100 Gy(10) for Non-bone Oligometastases(ELSEVIER INC, 2022-01-01) Kutuk, Tugce; Herrera, Robert; Mustafayev, Teuta Z.; Gungor, Gorkem; Ugurluer, Gamze; Atalar, Banu; Kotecha, Rupesh; Hall, Matthew D.; Rubens, Muni; Mittauer, Kathryn E.; Contreras, Jessika A.; McCulloch, James; Kalman, Noah S.; Alvarez, Diane; Romaguera, Tino; Gutierrez, Alonso N.; Garcia, Jacklyn; Kaiser, Adeel; Mehta, Minesh P.; Ozyar, Enis; Chuong, Michael D.Purpose: Randomized data show a survival benefit of stereotactic ablative body radiation therapy in selected patients with oligometastases (OM). Stereotactic magnetic resonance guided adaptive radiation therapy (SMART) may facilitate the delivery of ablative dose for OM lesions, especially those adjacent to historically dose-limiting organs at risk, where conventional approaches preclude ablative dosing. Methods and Materials: The RS Search Registry was queried for OM patients (1-5 metastatic lesions) treated with SMART. Freedom from local progression (FFLP), freedom from distant progression (FFDP), progression-free survival (PFS), and overall survival (LS) were estimated using the Kaplan-Meier method. FFLP was evaluated using RECIST 1.1 criteria. Toxicity was evaluated using Common Terminology Criteria for Adverse Events version 4 criteria. Results: Ninety-six patients with 108 OM lesions were treated on a 0.35 T MR Linac at 2 institutions between 2018 and 2020. SMART was delivered to mostly abdominal or pelvic lymph nodes (48.1\%), lung (18.5\%), liver and intrahepatic bile ducts (16.7\%), and adrenal gland (11.1\%). The median prescribed radiation therapy dose was 48.5 Gy (range, 30-60 Gy) in 5 fractions (range, 3-15). The median biologically effective dose corrected using an alpha/beta value of 10 was 100 Gy10 (range, 48-180). No acute or late grade 3+ toxicities were observed with median 10 months (range, 3-25) follow-up. Estimated 1-year FFLP, FFDP, PFS, and OS were 92.3\%, 41.1\%, 39.3\%, and 89.6\%, respectively. Median FFDP and PFS were 8.9 months (95\% confidence interval, 5.2-12.6 months) and 7.6 months (95\% confidence interval, 4.5-10.6 months), respectively. Conclusions: To our knowledge, this represents the largest analysis of SMART using ablative dosing for non-bone OM. A median prescribed biologically effective dose of 100 Gy10 resulted in excellent early FFLP and no significant toxicity, likely facilitated by continuous intrafraction MR visualization, breath hold delivery, and online adaptive replanning. Additional prospective evaluation of dose-escalated SMART for OM is warranted. (C) 2022 The Author(s). Published by Elsevier Inc. on behalf of American Society for Radiation Oncology.Item False Positive 18F-FDG Uptake in Mediastinal Lymph Nodes Detected with Positron Emission Tomography in Breast Cancer: A Case Report(HINDAWI LTD, 2013-01-01) Ugurluer, Gamze; Kibar, Mustafa; Yavuz, Sinan; Kuzucu, Akin; Serin, MeltemBreast cancer is the most frequently diagnosed cancer among females. It is accepted that lymph node involvement with metastatic tumor and the presence of distant metastasis are the most important prognostic factors. Accurate staging is important in determining prognosis and appropriate treatment. Positron emission tomography with computed tomography detectsmalignancies using 2-{[}18F]-fluoro-2-deoxy-d-glucose (18F-FDG PET CT) with high accuracy and they contribute to decisions regarding diagnosis, staging, recurrence, and treatment response. Here, we report a case of false positive metastatic mediastinal lymph nodes that were diagnosed by 18F-FDG PET CT in a 40-year-old breast cancer patient who had undergone preoperative evaluation. Right paratracheal, prevascular, aorticopulmonary, precarinal, subcarinal, hilar, and subhilar multiple conglomerated mediastinal lymph nodes were revealed in addition to left breast mass and axillary lymph nodes. Mediastinoscopy was performed with biopsy and pathology was reported as granulomatous lymphadenitis. In conclusion, any abnormal FDG accumulation in unusual lymph nodes must be evaluated carefully and confirmed histopathologically.Item Correlation between 18F-FDG Positron-Emission Tomography 18F-FDG Uptake Levels at Diagnosis and Histopathologic and Immunohistochemical Factors in Patients with Breast Cancer(AVES, 2016-01-01) Ugurluer, Gamze; Yavuz, Sinan; Calikusu, Zuleyha; Seyrek, Ertugrul; Kibar, Mustafa; Serin, Meltem; Ersoz, Canan; Demircan, OrhanObjective: In this study, we aimed to determine the correlation between pretreatment-staging 18F-FDG total body positron-emission tomography/computed tomography (PET/CT) maximum standardized uptake value (SUVmax) levels and histopathologic and immunohistochemical predictive and prognostic factors in patients with breast cancer. Materials and Methods: One hundred thirty-nine women with breast cancer who were treated between 2009 and 2015 at our hospital and who had pretreatment-staging PET/CT were included in the study. SUVmax levels and histopathologic and immunohistochemical results were compared. Results: The median age was 48 years (range, 29-79 years). The mean tumor diameter was 33.4 mm (range, 7-120 mm). The histology was invasive ductal carcinoma in 80.6\% of the patients. In the univariate analysis, SUVmax levels were significantly higher in patients with invasive ductal carcinomaItem Computed tomography based evaluation of prostatic fiducial marker migration between the periods of insertion and simulation(AVES, 2017-01-01) Arpaci, Taner; Ugurluer, Gamze; Ispir, Emine Burcin; Eken, Alper; Akbas, Tugana; Serin, MeltemObjective: The aim of this study was to determine whether significant fiducial marker migration occurs between the periods of prostatic marker insertion and computed tomography (CT) performed for radiotherapy planning and if a waiting period is necessary. Material and methods: Thirty-nine patients with prostate adenocarcinoma underwent fiducial marker insertion before radiotherapy between June 2013 and December 2015. Three markers were inserted by one radiologist under the guidance of transrectal ultrasonography. All patients underwent CT three hours after insertion to confirm the number and position of fiducial markers. Radiotherapy planning CT was performed on an average of 11 days (range 7-20) after insertion. CT images were imported into treatment planning system to analyze the position of fiducial markers. Point-based marker match algorithm was used to find the distance of marker migration. The mean and maximum distances between each fiducial markers were calculated. Results: The mean distance of migration was 1.029+/-0.42 mm (range 0.23-1.93 mm) and the maximum distance was 1.361+/-0.59 mm (range 0.25-2.74 mm). The distance of marker migration was not statistically significant for the groups organized according to the timing of marker insertion, prostate volume, patient age, prostate specific antigen level and Gleason score. Conclusion: According to our results significant fiducial marker migration did not occur during the interval between insertion and treatment planning CT. It should be taken into consideration that performing simulation on the same day as marker insertion might prevent increased cost and delayed radiation therapy by saving the patients from extra visits to the clinic.