bluuebunny/crossref_metadata_2025_split huggingface dataset:
Immunological and mutational characterization of non-uterine leiomyosarcoma: A pilot study.
Authors: Seliga Katarzyna , Teterycz Pawel , Szumera-Cieckiewicz Anna , Tysarowski Andrzej , Wagrodzki Michal , Kosela Hanna Melania , Kozak Katarzyna , Goryn Tomasz , Siedlecki Janusz A , Rutkowski Piotr 
Published: March 2020

e22529 Background: Data from many studies in cancer studies suggest that factors such as expression of PD-L1, tumor infiltrating lymphocytes (TILs) and high mutational burden (TMB) in the tumor cells are connected with better response to immune check-point blockage and may have prognostic value. Little is known about those factors in other neoplasms. Methods: In this retrospective study, we have included 31 patients with leiomyosarcoma (LMS) localized on extremity (21) or in retroperitoneal space (10) treated in our center between 2002-2018 with available biopsy specimens obtained before treatment. Mean age was 58 year (SD: 17). Male:Female ratio was 0.82. 14(45%) lesions were deemed high-grade sarcomas. 3 patients had metastases at the time of diagnosis. Immunohistological assays for TILs, tumor associated macrophages (TAMs), PD-1 and PD-L1. TMB was assessed in Tumor Mutational Burden Panel in the formalin-fixed paraffin-embedded samples. 7 cases were excluded from TMB analysis due to poor specimens quality. Fisher exact test was used to compare discrete variables while Kaplan-Meier estimate and stratified log-rank test were used to examine impact of explored factors on survival. Results: The median overall survival in this cohort was 33.4 months (95% confidence intervals, CI: 32-NA). TMB > = 10 mutations/Mb was present in 33% high-grade (HG) and in 25% cases of low-grade (LG) LMS (p = 0.067). PD-L1 expression was present in 54% of HG lesions and in 31% of LG LMS (p = 0.43). TILs and TAMs were highly correlated and present in 40% and 50% of HG LMS and LG LMS respectively (p = 0.722). Among explored factors only TILs and TAMs had significant influence on survival as deemed by log-rank test stratified by tumor grade (p = 0.004) with 3-year survival rate of 100% in groups without TILs/TAMs regardless of grade and 0% in HG TILs/TAMs(+) group and 60% (95% CI: 31-100%) in LG TILs/TAMs(-) group. Conclusions: Significant percentage of LMS exhibit immunohistological and molecular traits which potentially are connected with good response to check-point blockage agents. The immunological infiltration of primary LMS may be strong negative prognostic factor, what is interesting in sarcoma. Future expansion of this study cohort is warranted.
Renal Ewing's sarcoma: A single institute experience of seven patients.
Authors: Shishak Sorun , Rastogi Sameer , Panday Rambha , Sharma Dayanand , Barwad Adarsh , Aggarwal Aditi , Dhamija Ekta , Gupta Anshul , Balan Soorej , Deo Svs , Dutta Rimlee , Manasa Parisa , Kumar C Santosh , Kumar Pankaj 
Published: March 2020

e22538 Background: Renal Ewing’s sarcoma constitutes a rare subset of extraskeletal ewings with aggressive clinical course and dismal outcomes. The purpose of our study was to review our experience in the management of patients with Renal Ewing’s Sarcoma. Methods: We retrospectively analysed patients with Renal Ewing’s Sarcoma who were registered in Sarcoma Medical Oncology Clinic at AIIMS, New Delhi between September 2016 and January 2019. Results: A total of eight patients were registered in the study period, one of which was diagnosed with wilms tumor post nephrectomy and was excluded from our study. Of seven patients included in analysis, five (71.4%) were males. The commonest symptoms were flank pain (57.1%) and hematuria (57.1%). The median age was 28 years (range, 18 to 50 years). The mean Hemoglobin was 10.4 g/dl (range, 6-14 g/dl). The median tumor size was 13 cm (range, 5-25 cm). Four patients (57.1%) were non-metastatic while three (42.9%) had metastases in bone, lung and liver, and peritoneum respectively. Diagnosis was established by an upfront nephrectomy in four patients (57.1%) and biopsy in three patients (42.9%). Translocation for EWSR1 was done in 5 patients of which four (80%) were positive and one patient (20%) were negative. The test was not carried out in two patients due to inadequate tissue sampling. Three patients received neoadjuvant chemotherapy with VAC and the rest (four) received it after surgery. Ifosfamide-etoposide (IE) was added to VAC regimen in first two patients, in one being added post nephrectomy. This patient developed Ifosfamide-induced renal failure resulting in death. Radiotherapy was given in two patients, one pre-operative owing to large inoperable mass, and post operative in the other due to positive margins. At last follow up, three patients (42.9%) are alive and are on chemotherapy after radical nephrectomy. Four patients (57.1%) have died, three due to disease (all metastatic) and one due to chemotoxicity. The median survival was 15 months. Conclusions: Unlike extremity, renal ewings sarcoma more commonly presents in higher age and with advanced disease. Translocation studies are a prerequisite in a suspected case of Renal Ewings Sarcoma, especially due to possibility of discrepancies in pre and post-operative diagnoses. Our institutional protocol does not recommend the use of Ifosfamide in the subset of patients with single kidney, post nephrectomy. Caution must be exercised before instituting the drug and should be given only pre-operatively in absence of renal dysfunction.
Angiosarcoma treated with checkpoint inhibitors: A single-institution experience.
Authors: Florou Vaia , Wilky Breelyn A. , Trent Jonathan C. 
Published: March 2020

e22543 Background: Angiosarcoma (AS) is a rare endothelial malignancy with only 2–3 new cases per one million diagnosed yearly but is among the most aggressive soft tissue sarcomas. Some cutaneous angiosarcomas may have a mutational landscape similar to UV light exposed melanoma. These findings offer a rationale for the early limited evidence of checkpoint inhibitor activity in cutaneous angiosarcomas. Methods: This is a retrospective review of patients with locally advanced or metastatic angiosarcoma, who were treated with checkpoint inhibitors at our institution. Descriptive statistics were used to analyze our findings. Results: Since 2016, we have treated seven angiosarcoma patients with checkpoint inhibitors either in the context of clinical trials or off label [Pembrolizumab + Axitinib (NCT02636725; n = 1), a CTLA-4 inhibitor (NCT02694822; n = 2), Pembrolizumab (n = 4)]. Five patients had cutaneous AS, one primary breast AS and one radiation associated breast AS. The average age was 66 years old and 5/7 (71%) patients were females. All patients had received prior systemic therapies (range 1-7, mean 3) and 2/7 (29%) patients had metastatic disease at the time of treatment. The mean number of checkpoint inhibitor doses was 6. At twelve weeks, 5/7 patients (71%) had partial response, by RECIST, of their lesions either on imaging or clinical exam and two (29%) had progressive disease. 6/7 patients are alive to date and, thus far, 3/7 patients (43%) progressed (median 3.4 months)- one achieved partial response after pembrolizumab was switched to ongoing Nivolumab/Ipilimumab, one died of progressive disease at 31 weeks (primary breast AS) and one was placed on subsequent therapy. No patient experienced any toxicities more than grade 2. Conclusions: This is a single institution, retrospective review of patients with AS treated with checkpoint inhibitors, first reported in the literature. Checkpoint inhibitors may be an effective and safe treatment option for this aggressive sarcoma. Prospective clinical trials are needed to systemically study this effect.
Activity of chemotherapy in inflammatory myofibroblastic tumor (IMT): A retrospective analysis within the Italian Rare Tumours Network (RTR).
Authors: Baldi Giacomo Giulio , Gronchi Alessandro , Vincenzi Bruno , De Pas Tommaso Martino , Pantaleo Maria A. , D'Ambrosio Lorenzo , Grignani Giovanni , Casanova Michela , Ferrari Andrea , Simeone Noemi , Provenzano Salvatore , Dei Tos Angelo Paolo , Sbaraglia Marta , Collini Paola , Dagrada Gianpaolo , Morosi Carlo , Casali Paolo Giovanni , Stacchiotti Silvia 
Published: March 2020

e22545 Background: IMT is a rare soft tissue tumour often curable with surgical resection. Nonetheless, in advanced cases a medical therapy is needed. Few data are available on the role of conventional chemotherapy. We report on a retrospective study on the activity of cytotoxic chemotherapy in pts with IMT treated within the RTR. Methods: Patients (pts) of any age with a centrally confirmed diagnosis of IMT treated with anthracycline-based or methotrexate (MTX) and vinorelbine/vinblastine-based regimens between 2008 and 2018 were reviewed. ALK status was evaluated by immunohistochemistry and FISH. Response was assessed by RECIST 1.1. Results: Twenty pts were retrospectively identified (ALK positive = 12, under assessment = 8); median age at the time of treatment start = 38, range = 5-77; disease extent: local disease = 2, locally advanced = 9, metastatic disease = 9. Thirteen pts (65%) received an anthracycline-based regimen, 7 (35%) MTX and vinorelbine/vinblastine. In the anthracycline-based group, 6 (46%) and 7 (54%) pts were treated for localized and advanced disease, respectively. Twelve pts were evaluable for response. Best responses were: 1 CR (8%), 6 PR (50%), 2 SD (17%), 3 PD (25%), for an ORR = 58%. 8 pts received a complete surgical resection at the end of the medical treatment and 6 are disease free. At 39 mos median follow-up, mPFS for 7 pts treated for advanced disease was 5.4 mos (IQR: 1.9-18.8). In the MTX and vinorelbine/vinblastine group, 2 (28%) and 5 (72%) pts were treated for localized disease and advanced disease, respectively. All pts were evaluable for response. Best responses were: 5 PR (50%), 3 SD (30%), 2 PD (20%), for an ORR = 50%. Two pts received a complete surgical resection after the end of chemotherapy and both are disease free. At 56.4 mos median follow-up, mPFS for pts with advanced disease was not reached, with disease progression reported only in 1 pt. Conclusions: Chemotherapy is an option in localised IMT when tumour shrinkage is needed as well as for tumour control in advanced disease. Clearly, its efficacy should be compared with ALK inhibitors, although of course both groups of agents may be resorted to stepwisely in advanced cases in need for medical therapy.
Clinical benefit of next generation sequencing in soft tissue and bone sarcoma: Rush University Medical Center’s experience.
Authors: Weiss Mia C. , Blank Alan , Gitelis Steven , Fidler Mary J. , Batus Marta 
Published: March 2020

e22552 Background: The overall survival for metastatic sarcoma has remained at only 18-20%. In the era of next generation sequencing (NGS), much research is ongoing on identifying optimal treatments. The MULTISARC trial aims to determine if NGS can lead to improved overall survival by randomizing patients with metastatic STS to receive NGS (followed by possible NGS-guided therapy) or not. We present our center’s experience with NGS in sarcomas patients. Methods: Patients with soft tissue and bone sarcomas at Rush that had the Foundation Medicine assay sent on tumor samples between August 2017 and August 2018 were analyzed retrospectively. The impact of NGS on clinical decision making was determined based on patients being prescribed off-label FDA-approved therapy targeting identified mutation. Results: Thirty-four patients with bone/soft tissue sarcomas that had NGS sent on specimens were identified. Median age at diagnosis: 43 (18-78 years); 18 males, 16 females. Histologic subtypes: synovial sarcoma, myxofibrosarcoma, leiomyosarcoma, chondrosarcoma, sclerosing epitheloid fibrosarcoma, PEcoma, pleomorphic undifferentiated sarcoma, MPNST, liposarcoma- well and de-differentiated, angiosarcoma, osteosarcoma. 16/34 patients had targetable mutations with approved therapies in tumor types other than sarcoma. Four of these patients had therapy changed based on NGS results, 1 patient with metastatic chondrosarcoma (PTEN mutation, everolimus added), 1 patient with metastatic liposarcoma (CDK4 mutation, palbociclib added), 1 patient with metastatic osteosarcoma (CCD1/CDK4 and a PDGFRA mutation for which palbociclib followed by imatinib was added), and 1 patient with metastatic pleomorphic undifferentiated sarcoma (CDK4 mutation, palbociclib added). Targetable mutations for which clinical trials are available were identified in 25/34 (73%) of the cases. Conclusions: NGS was readily able to identify actionable mutations in close to 50% of patients with clinical trial opportunities in close to 75%. Four patients had therapy changed as a result of NGS testing. Although our study size is small, our data show potential for the use of genomic profiling to identify actionable targets, tailor therapy, and hopefully improve outcomes. [Table: see text]
Efficacy of low dose pazopanib in advanced soft tissue sarcoma (STS): Experience from a dedicated sarcoma medical oncology clinic in India.
Authors: Kumar C Santosh , Rastogi Sameer , Prajapati Ramjas , Barwad Adarsh , Aggarwal Aditi , Dhamija Ekta , Panday Rambha , Gupta Anshul , Dutta Rimlee , Deo SVS , Manasa Parisa , Kumar Pankaj , Shishak Sorun 
Published: March 2020

e22555 Background: The approved dose of pazopanib in advanced STS is 800 mg/day and is not weight based. After initial patients who couldn’t tolerate pazopanib we started pazopanib at lower doses escalating to higher doses. There is already preliminary data about low dose pazopanib in asian patients. Methods: We retrospectively analysed the prospectively kept data base of patients with advanced non adipocytic STS who were treated with pazopanib at AIIMS sarcoma medical oncology clinic between September 2015 and February 2019. After start of pazopanib we get CT scan done every 3 monthly as institutional protocol. Statistical analysis was done by SPSS 23. Results: There were total of 66 patients with median age 40 yrs (17 – 81)and majority were males who (60). ECOG Performance status (PS) was PS 1 in 63% and PS 2 in 23%. Most common diagnosis was synovial sarcoma (22%), leiomyosarcoma (18 %), angiosarcoma (9%), MPNST(8%) and others (43%). Most of the patients received pazopanib as second line (59%) and third line (31%). 90% of patients received dose of 400 mg and remaining 10% of the patients received 600 mg of dose (in which it could be escalated after 1 month). Median weight was 63kg and median height in our patients was 160cm while the median BSA was 1.6. Dose escalation was attempted, but was not feasible due to toxicities. Out of total 66 patients, 57 were available for response assessment. RECIST responses were complete response (CR) was present in 2.9%, partial response (PR) in 17% , stable disease (SD) in 28%. The median progression free survival (PFS) was 5 months and median overall survival was 18 months after a median duration of follow up of 20 months. Grade 3 and grade 4 toxicities were present in 31% of the patients. Grade 3 hand foot syndrome (HFS) was present in 12% of patients.4.5% had hypertension. Other grade 3 and 4 toxicities were oral mucositis (6%) , transaminitis (4%) Cardiomyopathy (3%) and cholestasis jaundice (1.5%) with bilirubin elevated to the level on 19.1 mg/dl. This resolved after stoppage of Pazopanib. Conclusions: Pazopanib at low doses in our series was as efficacious and with similar toxicity as previous literature The option of lower dose pazopanib needs to be explored in Asian population who have lower body weight.
Prevalence of symptom clusters among sarcoma patients in active treatment.
Authors: Copeland Vanessa C. , Phun Jennifer , Segal Eve M. , Hammer Kathryn J. , Loggers Elizabeth T. 
Published: March 2020

e22561 Background: Cancer patients (pt) frequently experience symptoms from cancer or its treatment. Two or three co-occurring, uncontrolled symptoms (symptom clusters, SC), can worsen cancer pt morbidity and mortality. Although SC have been characterized in other cancer pts, little research has been conducted in sarcoma pts. Methods: SC were studied via retrospective chart review of adult sarcoma pts receiving treatment between 09/2018-01/2019 in a dedicated sarcoma clinic. Pts completed a 33-item, modified Patient Reported Outcomes-Common Terminology Criteria for Adverse Events (mPRO-CTCAE)-based review of systems (hereafter “form”) for ongoing clinical care with presence of individual symptoms defined as ≥“occasionally.” Demographic, disease and treatment specific data was collected; descriptive data (%’s) are presented. Results: Pts (n = 153 completing 321 forms) were median 57 years (range 20-81), 48% male, 68% white, with lower extremity (29%) or abdomen (46%) sarcoma with 67% receiving IV chemo (33% oral chemo), with the majority at full dose (77%). Most had leiomyosarcoma 18%, liposarcoma 14%, GIST 12%, or NOS 24% with 59% metastatic disease. Five SC triads occurring in > 25% of pts who fully completed one form were identified (see Table). Eighty-one pts completed 2 or more forms (mean 26.7 days from first to second form [std dev 17.7, range 2-77]) with 50-67% of evaluable pts having ongoing SC triads. Conclusions: Symptom clusters are common among sarcoma patients on active treatment. Future research should address optimal management of these clusters. Additional data, including severity/interference of symptoms, longitudinal changes and predictors of SC, will be presented.[Table: see text]
Bridging the information gap: A scoping review of radiation oncology patient education scholarship.
Authors: Rosenberg David M. , Rooney Michael Kevin , Arya Ritu , Abrams Matthew J. , Braunstein Steve E. , Croke Jennifer M. , Giuliani Meredith Elana , Gunther Jillian Rebecca , Hirsch Ariel , Ingledew Paris-Ann , Turner Sandra , Golden Daniel William 
Published: March 2020

e23164 Background: Patient education (PE), the use of educational interventions to improve patient knowledge of healthcare, has been shown to reduce patient anxiety and facilitate shared decision making. In contrast to many other medical specialties, the state of radiation oncology (RO) PE is unclear. This scoping review aims to characterize the state of ROPE peer-review scholarship to describe strengths, identify gaps and guide future ROPE research. Methods: Predetermined medical subject headings and search terms were used to search PubMed for ROPE articles. Additional terms were identified from these articles and the search was repeated until no further articles were found. In-press articles in major RO and medical education (ME) journals were searched. English language articles published after 1/1/1990 that had a primary ROPE focus were included. Two independent reviewers performed the search and determined eligibility. Articles were categorized using the most predominant step of Kern’s six-step conceptual framework for ME curriculum development and the country and year of publication. Results: Of 11,756 identified articles, 182 met inclusion criteria. Articles per year increased over time (p < 0.01): 1990-1994 (5.5%); 1995-1999 (11.0%); 2000-2004 (13.7%); 2005-2009 (10.4%); 2010-2014 (25.8%); 2015-2019 (33.5%). Conceptual framework categorization was as follows: problem identification (22.0%); needs assessment (23.6%); goals and objectives (4.9%); education strategies (9.9%); implementation (7.7%); evaluation and feedback (31.9%). Regional breakdown was as follows with some articles spanning regions: Africa (1.1%); South America (2.2%); Asia (6.0%); Australia (15.4%); Europe (30.2%); North America (45.6%). Conclusions: Nearly half of articles are primarily problem identification or needs assessment (45.6%) which are descriptive in nature and only identify or clarify ROPE needs. Additionally while these results are limited to English language scholarship, there are significant regional maldistributions. Although there is an encouraging trend of increasing ROPE publications over time, this study identifies opportunities to further develop, implement and evaluate ROPE scholarship.
What type of information is needed by AYA cancer survivors in Japan?
Authors: Yamaki Chikako , Nakatani Yuki , Sato Toshiko , Taniguchi Hikaru , Takayama Tomoko 
Published: March 2020

e23165 Background: Cancer patients and survivors in the adolescents and young adults (AYA) generation often have a wide range of unmet needs, which include health related matters, communication problems, relationship difficulties, and life roles. Japanese AYA cancer survivors supposedly have these same unmet needs, but it is not clear which specific types of needs should be provided. The purpose of this study was to explore the support needs of Japanese AYA cancer survivors and to propose what type of information they should be provided. Methods: Eleven AYA cancer survivors and their families participated in group interviews. All participants were recruited from the Cancer-Civil Panel of Center for Cancer Control and Information Services, National Cancer Center Japan (NCC-CIS), which is commissioned by the NCC president to support NCC-CIS activities. Participants were asked about their 1) difficulties and painful issues during their cancer history, 2) information needs to handle those difficulties, and 3) willingness to support AYA patients suffering from cancer. Interviews were audio recorded and transcribed verbatim. Three researchers coded the transcripts and constructed the categories to be assessed. Results: Difficulties and painful issues were compiled into four categories: (1) physical and psychological pain that come directly from cancer, (2) uncertainty of the situation and the future, (3) isolation and alienation that come from his/her unique experiences, and (4) distress of facing painful families. Information and support needs included three categories: (1) detailed experiences from the patient’s view, (2) ways to face his/her cancer and life role as a survivor, and (3) role models or companies for sharing the same experiences. All participants expressed the willingness to support someone by the disclosure of their own experiences. Conclusions: Japanese AYA survivors had unmet information needs for both medical and lifelong psycho-social issues. They would like to have information about a variety of personal and subjective experiences in order to recommend possible future solutions. These findings suggest that personal and narrative experiences would support AYA survivors in their efforts to confront their uncertain future with cancer.