tbuckley/cabot-search huggingface dataset:
63. Immunogenicity of Self Tumor Associated Antigens Is Enhanced Through Protein Truncation
Authors: Tim Kottke, Kevin G. Shim, Laura Evgin, Vanesa Alonso-Camino, Shane Zaidi, Rosa Diaz, Jose Pulido, Jill Thompson, Karishma Rajani, Amanda Huff, Elizabeth Ilett, Hardev Pandha, Kevin Harrington, Peter Selby, Alan Melcher, Richard Vile
Journal: Molecular Therapy (2016)

We showed previously that expressing tumor-associated antigens (TAA) from Vesicular Stomatitis Virus (VSV) eradicates established tumors. We show here that truncation of TAA expressed from VSV can occur to preserve the ability of the virus to replicate efficiently. We observed that truncation of VSV-expressed TAA affects the processing of the antigen, causing a bias towards an IL-17 anti-tumor response which was raised by cumulative signaling from different types of APC, each presenting specific, truncated antigens. Whereas processing of full length, self-TAA invoked an IFN-γ based, CD8+ dependent response, truncated versions of the same self-TAA (likely to be poorly and incompletely folded) were processed through a class II-dependent pathway, and invoked an IL-17 based response. Significantly, the IL-17-mediated anti tumor response was both more therapeutic, and durable, than the response against full length self-TAA. These data show that the type/potency of anti-tumor immune responses against self-TAA can be manipulated in vivo through the nature of the self protein (full length or truncated), inclusion of multiple TAA to recruit the optimal combination of APC, and the resultant skewing of the T cell response to either an IFN-γ or IL-17 producing phenotype. Therefore, in addition to generation of neoantigens through sequence mutation, immunological tolerance against self-TAA can be broken through manipulation of protein integrity, allowing for rational design of better self immunogens for cancer immunotherapy.
Combination of Adoptive Cellular Immunotherapy and Bortezomib-Based Regimens Increase the Progression Free Survival of the Patients with Multiple Myeloma
Authors: Jiuwei Cui, Wei Li, Lei Qian, Chunshui Liu, Haofan Jin, Ou Bai, Yanqiu Song, Chao Niu, Dan Li, Huimin Tian, Wei Han, Hua He, Wei Song
Journal: Blood (2014)
508P Prognostic factors of soft tissue sarcoma (STS) treated with pazopanib from Nishinomiya Sarcoma Cohort Study (NSCS)
Authors: H. Narahara, E. Tanaka, M. Morimoto, K. Morita, J. Fukushima, S. Iio, Y. Yasunaga, Y. Inui, S. Kawata, K. Takahashi
Journal: Annals of Oncology (2016)
403TiP CREAM study: Clinical correlation between ipilimumab-Related colitis And intestinal Microbiote in metastatic melanoma patients
Authors: L. Orgiano, A. Dessi, A. Cubeddu, E. Lai, R. Mascia, S. Tolu, V. Palmas, T. Camboni, C. Madeddu, E. Massa, G. Astara, A. Manzin, M. Scartozzi
Journal: Annals of Oncology (2016)
Construction of a viral T2A-peptide based knock-in mouse model for enhanced Cre recombinase activity and fluorescent labeling of podocytes
Authors: Sybille Koehler, Sebastian Brähler, Fabian Braun, Henning Hagmann, Markus M. Rinschen, Martin R. Späth, Martin Höhne, F. Thomas Wunderlich, Bernhard Schermer, Thomas Benzing, Paul T. Brinkkoetter
Journal: Kidney International (2017)
<i>PTPRF </i>expression as prognostic/predictive marker for treatment with erlotinib in non-small cell lung cancer (NSCLC)?
Authors: Barbara Klughammer, Walter Bordogna, Paul Delmar
Journal: Journal of Clinical Oncology (2012)

22 Background: Erlotinib is an EGFR TKI established as maintenance, second- and third-line treatment in unselected NSCLC patients and as first-line treatment in patients harboring EGFR mutations. Aberrations in the receptor type protein tyrosine phosphatase (PTP) gene family were described by Jacob and Motiwala (2005) as a hallmark of carcinogenesis. Recent research established the role of PTPRS, a member of the same gene family, as necessary for the dephosphorylation of EGFR in head and neck cancer (Morris and Bivona. 2011). The role of PTPRF as an interesting prognostic marker in NSCLC and potential predictive marker for treatment with erlotinib is reported here. Methods: MERIT was a single arm phase II clinical trial (Tan et al. 2010) where all patients had biopsies performed by bronchoscopy before starting treatment with erlotinib (150 mg/day p.o.). Biopsies were snap frozen and RNA was extracted using laser capture microdissection to enrich for tumor content. Affymetrix Human U133A microarrays were used to analyse gene expression profiles. Exploratory statistical methods identified several gene candidates by comparing benefitting and nonbenefitting patients including PTPRF. These findings were confirmed by qRT-PCR in both fresh and FFPE tissue. Here we describe analysis of PTPRF in FFPE tissue from the randomized phase III SATURN trial (Cappuzzo et al. 2010) Results: PTPRF expression was found to be greater in benefitting patients than in nonbenefitting patients in MERIT HR 0.50 (0.34 - 0.73), p = 0.0004 on a continuous log2 scale. Results from SATURN further suggested predictive characteristics for PTPRF, with a larger treatment effect observed in patients with high levels as defined above median. Conclusions: Data from MERIT and SATURN suggest PTPRF to be a possibly prognostic marker for OS in NSCLC and potential predictive marker for treatment with erlotinib. This gene family is very interesting and further research, especially MoA is warranted. [Table: see text]
MNGO-07. TREATMENT AND PROGNOSIS IN ADULT PATIENTS WITH MALIGNANT SPINAL CORD MENINGIOMA
Authors: Kailin Yang, Leo Kim, John Suh, Erin Murphy, Samuel Chao
Journal: Neuro-Oncology (2016)

Spinal cord meningioma is the most common primary spinal cord tumor. The prognosis and treatment response for the subset with malignant meningioma are not well characterized. We analyzed 67 adult patients of age 19 or older with the diagnosis of malignant meningioma in the spinal cord identified from the Surveillance, Epidemiology, and End Results database (1973-2012). Kaplan-Meier analysis and multivariate Cox proportional hazard modeling were used to assess the impact of treatment on overall survival (OS). The median age was 61 years with 60-74 as the peak age group (37.3%). 25.3% of patients received radiotherapy (RT). Surgery was only performed in a subset of patients with 10.5% receiving sub-total resection (STR) and 20.9% receiving gross total resection (GTR). OS at 10 years was 56.3%, with a median follow-up time of 11.4 years. The most common histological diagnosis was malignant meningioma (9530/3, 94.0%), with the rest of malignant hemangioblastic meningioma (9535/3, 4.5%) and papillary meningioma (9538/3, 1.5%). Female gender was associated with better OS at 10 years, with 71.7% for female and 33.3% for male (p<0.01). Multivariate analysis adjusting for gender, year of diagnosis, treatment regimens showed worse OS in advanced age group 75+ (HR 3.120, 95% CI 1.020-10.744, p=0.046, using age group 18-44 as reference). However, there was no association of statistical significance with OS for the delivery of RT (HR 1.767, 95% CI 0.636-4.772, p=0.27) or surgery such as STR (HR 2.670, 95% CI 0.680-8.915, p=0.15). Adult patients with malignant meningioma were predominantly female, which was associated with improved OS compared to male gender. Long-term OS was optimal, and there was no clear evidence of RT or surgery affecting survival outcome. Given the potential selection bias and lack of detailed information on chemotherapy and RT dose in SEER database, further study is needed to validate our findings.
Sirolimus and Mycophenolate Mofetil as Graft-Versus-Host Disease Prophylaxis in Myeloablative, Matched Related Donor Hematopoietic Cell Transplantation
Authors: Laura Johnston, Mareike Florek, Randall Armstrong, Jeannine S McCune, Sally Arai, Ginna Laport, Robert Lowsky, David Miklos, Judith Shizuru, Wen-Kai Weng, Kevin Sheehan, Philip Lavori, Robert Negrin
Journal: Blood (2008)
A Case of Hypersensitivity to Mosquito Bites with Type III Latency of EB Virus Infection in NK Cells with Prolonged Survival Due to Induction of Specific CD8+ Cytotoxic T Cells.
Authors: Shigeru Tsuchiya, Wei Du, Hideaki Kikuta, Toru Uchiyama, Masaei Onuma, Takeshi Rikiishi, Yoji Sasahara, Miyako Yoshinari Ohuchi, Masayoshi Minegishi, Satoru Kumaki
Journal: Blood (2006)
Development of medium throughput cell-based ELISAs to quantitate the activity of PI3K-AKT pathway inhibitors
Authors: tao wang, Lillian Sams, Sandaruwan Geeganage
Journal: Cancer Research (2007)

AACR Annual Meeting-- Apr 14-18, 2007; Los Angeles, CA

290 

PI3K/AKT signaling pathway controls cell survival, cell-cycle progression, cell growth and metabolism through a cascade phosphorylation of a number of key substrates. This pathway is regulated by three well characterized tumor suppressors; pten, tsc2, and lkb1 . Deletion of these genes results in activation of the AKT pathway and proliferative disorders. In fact, many human tumors show activation of the PI3K/AKT pathway and therefore it has become an attractive pathway for modulation to develop targeted cancer therapies. This can be achieved by developing small molecule inhibitors that target PI3 Kinase, PDK1, AKT, mTOR, or S6K and assessed utilizing conventional biochemical assays. However, it is also necessary to understand the effects of these molecules on phosphorylation events in cell based systems. Currently a widely used method for evaluating these effects is by western blotting, a labour-intensive and limited capacity method which is not suitable for drug discovery. Therefore we developed a series of novel cell-based ELISAs to measure the extent of protein phosphorylation by employing phospho-specific antibodies in 96-well plate format in a number of different adherent cancer cell lines. These assays have been used to quantitate the inhibition of phosphorylated target proteins, including pGSK, pAKT, pmTOR and pS6 in several human tumor cell lines exposed to various stimuli and well characterized pathway inhibitors such as Rapamycin and LY294002. SARs can be developed utilizing these assays to evaluate inhibitors that are active on PI3K, PDK1, AKT, mTOR and p70S6K. These assays can also be utilized as primary screens in an automated high throughput setting to identify inhibitors that modulate the pathway by novel mechanisms.
Improved efficacy response attributed to diagnostic selection – Interim results of the phase 1 experience from ALKA-372-001
Authors: J. Christiansen, S. Siena, E. Valtorta, A. Johnson, D. Murphy, R. Shoemaker, J. Lamoureux, D. Luo, R. Patel, Z. Hornby, P. Multani, E. Chow Maneval, M. Duca, F. Debraud
Journal: Annals of Oncology (2016)
Next Generation DNA Sequencing of Novel Loci in Workers with Diisocyanate Asthma (DA)
Authors: Zana L. Lummus, Kenneth Kaufman, Matthew T. Weirauch, Andre Cartier, Maria-Jesús Cruz, Banu Kesavalu, Catherine Lemiere, Xavier Muñoz, Ignacio Perez-Camo, Santiago Quirce, Joaquin Sastre, Berran Yucesoy, David I. Bernstein
Journal: Journal of Allergy and Clinical Immunology (2017)
CMV Viral Replication Determined by Quantitative PCR and Pp-65 Antigenaemia after Allogeneic Hemopoietic Stem Cell Transplantation (ALO-HSCT)
Authors: Fáti ma de la Cruz Vicente, P. Perez-Romero, M. Aguilar-Guisado, R. Parody Ruiz-Berdejo, Jm. Cisneros-Herreros, A. Urbano-Ispizua, Ildefonso Espigado
Journal: Blood (2008)
Hevylite, a Novel M-Component Based Biomarkers of Response to Therapy and Survival in Waldenstrom Macroglobulinemia
Authors: Salomon Manier, Julie Lejeune, Lucile Musset, Eileen Boyle, Remy Dulery, Houria Debarri, Jean-Luc Faucompret, Jordan Gauthier, Claire Bories, Guillemette Fouquet, Charles Herbaux, Sabine Tricot, Annie Pietrantuono, Frederic Combat, Stephanie Poulain, Stephen Harding, Véronique Leblond, Xavier Leleu
Journal: Blood (2011)
Tuning Chromosomal Instability to Optimize Tumor Fitness
Authors: Mark E. Burkard, Beth A. Weaver
Journal: Cancer Discovery (2017)

Abstract Summary: Low rates of chromosomal instability (CIN) are weakly tumor promoting, whereas high rates of CIN cause cell death and tumor suppression. In this context, Sansregret and colleagues show that one mechanism to restrain excessive CIN in tumor cells and increase fitness is through mutations in the anaphase promoting complex/cyclosome. This serves to delay mitotic progression and decrease the rate of chromosome missegregation. Cancer Discov; 7(2); 134–6. ©2017 AACR. See related article by Sansregret et al., p. 218.
Elevated Circulating Tissue Factor Procoagulant Activity, Factor VII, Plasminogen Activator Inhibitor-1 and VCAM-1 in Childhood Obesity: Evidence of a Procoagulant State and Endothelial Dysfunction?,
Authors: Anamika Singh, Jay Gunawardana, Tara Alexis McCoy, Tina Nguyen, Stephanie Vander Veur, Treasure Stephens, Darnice Martinez, Eugene Komaroff, Gary D. Foster, A. Koneti Rao
Journal: Blood (2011)
Evaluation of the Prognostic Implications of Pro- and Anti-Angiogenic Cytokines in 311 Patients with Untreated Chronic Lymphocytic Leukemia.
Authors: Tait D. Shanafelt, S. Geyer, N. Bone, S. Schwager, Y. K. Lee, G. Nowakowski, T. G. Call, C. S. Zent, N. E. Kay
Journal: Blood (2005)
Early Central Nervous System Complications after Allogeneic Stem Transplantation: A Single-Center Analysis of 723 Patients Including 456 Cord Blood Recipients
Authors: Kosei Kageyama, Hisashi Yamamoto, Mitsuhiro Yuasa, Daisuke Kaji, Sachie Wada, Aya Nishida, Kazuya Ishiwata, Shinsuke Takagi, Masanori Tsuji, Go Yamamoto, Yuki Asano-Mori, Naoyuki Uchida, Koji Izutsu, Atsushi Wake, Akiko Yoneyama, Shigeyoshi Makino, Shuichi Taniguchi
Journal: Blood (2014)
Inflammatory Bone Loss Drives Skeletal Deterioration in a Murine Model of Sickle Cell Disease
Authors: David R. Archer, Prasad Mulupuri, Prasanthi Chappa, Tatyana Vikulina, Suzanne Roser-Page, Masayoshi Yamaguchi, Patrice L Capers, Hyacinth I Hyacinth, Jacqueline Hibbert, Neale Weitzmann
Journal: Blood (2011)
Relapse in Teenage and Young Adult (TYA) Patients Treated on a Pediatric Minimal Residual Disease (MRD) Stratified Protocol Is Associated with a Poor Outcome: Results from UKALL2003
Authors: Rob S Sellar, Clare Rowntree, Ajay J. Vora, Caroline L Furness, Nicholas Goulden, Chris Mitchell, Anthony V. Moorman, Rachael Hough
Journal: Blood (2015)
Defective Branched-Chain Amino Acid Catabolism Disrupts Glucose Metabolism and Sensitizes the Heart to Ischemia-Reperfusion Injury
Authors: Tao Li, Zhen Zhang, Stephen C. Kolwicz, Lauren Abell, Nathan D. Roe, Maengjo Kim, Bo Zhou, Yang Cao, Julia Ritterhoff, Haiwei Gu, Daniel Raftery, Haipeng Sun, Rong Tian
Journal: Cell Metabolism (2017)
First Report on the Safety and Efficacy of a Long-Acting Recombinant FVIII (turoctocog alfa pegol, N8-GP) during Major Surgery in Patients with Severe Hemophilia a
Authors: Kingsley Hampton, Pratima Chowdary, Scott Dunkley, Silke Ehrenforth, Lotte Jacobsen, Anne T. Neff, Elena Santagostino, Jameela Sathar, Hideyuki Takedani, Clifford M. Takemoto, Claude Négrier
Journal: Blood (2015)
Examining health beliefs: Why mastectomies are on the rise.
Authors: Andrea Marie Covelli, Nancy N. Baxter, Margaret Fitch, Frances Catriona Wright
Journal: Journal of Clinical Oncology (2014)

192 Background: Rates of unilateral (UM) and contralateral prophylactic mastectomy (CPM) for early-stage breast cancer (ESBC) have been increasing. To understand this increase we sought the perspectives of patients who chose UM+/-CPM and treating surgeons. Methods: Applying the Health-Belief Model, we completed a qualitative study examining the surgeon’s practice and patient’s decision-making that resulted in the choice for mastectomy. Purposive sampling identified women across Toronto, Canada who were candidates for breast conserving therapy (BCT) but underwent UM+/-CPM. Academic and community breast surgeons from across Ontario, Canada and the United States were also recruited. Data were collected through semi-structured interviews, this continued until data saturation was reached. Constant comparative analysis identified key ideas. Results: 29 patients and 45 surgeons completed interviews. The dominant theme was a ‘misperceived threat’. Despite surgeons describing the high survivability of ESBC, patients greatly overestimated the threat of ESBC and strived to eliminate this threat by choosing mastectomy. Surgeons described BCT and UM as equivalent treatment options for ESBC, and often recommended BCT. In this average-risk population CPM was discouraged by the surgeons describing no survival advantage; despite this, women requested UM+CPM. Personal cancer experiences with family and friends were extremely influential in women’s request for UM+/-CPM. Previous negative experiences translated into an overestimated risk of recurrence, contralateral cancer, metastasisand subsequent death. Despite evidence-based consultations, patients misperceived the risks and severity of ESBC, and believed that by choosing UM+/-CPM they would live longer. Most women did not perceive any risks of undergoing mastectomy, yet many had ongoing issues with disturbed skin sensation, cosmesis and body image. Conclusions: A previous cancer experience was influential in the choice for UM+CPM. Despite surgeons counseling otherwise, women greatly overestimated the risk of ESBC and misperceived the benefits of mastectomy. Improved discussion of patient sources of information and fears around survival would benefit surgical consultations.
Nab-paclitaxel as second line treatment in advanced gastric cancer: A HORG multicenter phase II study
Authors: P. Katsaounis, N. Kentepozidis, A. Kotsakis, A. Polyzos, L. Vamvakas, M. Bakogiorgos, I. Boukovinas, E. Hartabilas, E. Prinarakis, T. Skaltsi, V. Georgoulias, J. Souglakos
Journal: Annals of Oncology (2016)
Acceptability of the Fertility Preservation Program and Evaluation of the Gonadal Function in Children after Allogeneic Stem Cell Transplantation
Authors: Sabine Amah, Catherine Poirot, Sylvie Chevret, Mony Fahd, Karima Yakouben, Marie Ouachee, Jean Claude Carel, Andre Baruchel, Jean-Hugues Dalle
Journal: Blood (2015)
Augmentation of Muscle Blood Flow by Ultrasound Cavitation Is Mediated by ATP and Purinergic Signaling
Authors: J. Todd Belcik, Brian P. Davidson, Aris Xie, Melinda D. Wu, Mrinal Yadava, Yue Qi, Sherry Liang, Chae Ryung Chon, Azzdine Y. Ammi, Joshua Field, Leanne Harmann, William M. Chilian, Joel Linden, Jonathan R. Lindner
Journal: Circulation (2017)

Augmentation of tissue blood flow by therapeutic ultrasound is thought to rely on convective shear. Microbubble contrast agents that undergo ultrasound-mediated cavitation markedly amplify these effects. We hypothesized that purinergic signaling is responsible for shear-dependent increases in muscle perfusion during therapeutic cavitation.Unilateral exposure of the proximal hindlimb of mice (with or without ischemia produced by iliac ligation) to therapeutic ultrasound (1.3 MHz, mechanical index 1.3) was performed for 10 minutes after intravenous injection of 2×108 lipid microbubbles. Microvascular perfusion was evaluated by low-power contrast ultrasound perfusion imaging. In vivo muscle ATP release and in vitro ATP release from endothelial cells or erythrocytes were assessed by a luciferin-luciferase assay. Purinergic signaling pathways were assessed by studying interventions that (1) accelerated ATP degradation; (2) inhibited P2Y receptors, adenosine receptors, or KATP channels; or (3) inhibited downstream signaling pathways involving endothelial nitric oxide synthase or prostanoid production (indomethacin). Augmentation in muscle perfusion by ultrasound cavitation was assessed in a proof-of-concept clinical trial in 12 subjects with stable sickle cell disease.Therapeutic ultrasound cavitation increased muscle perfusion by 7-fold in normal mice, reversed tissue ischemia for up to 24 hours in the murine model of peripheral artery disease, and doubled muscle perfusion in patients with sickle cell disease. Augmentation in flow extended well beyond the region of ultrasound exposure. Ultrasound cavitation produced an ≈40-fold focal and sustained increase in ATP, the source of which included both endothelial cells and erythrocytes. Inhibitory studies indicated that ATP was a critical mediator of flow augmentation that acts primarily through either P2Y receptors or adenosine produced by ectonucleotidase activity. Combined indomethacin and inhibition of endothelial nitric oxide synthase abolished the effects of therapeutic ultrasound, indicating downstream signaling through both nitric oxide and prostaglandins.Therapeutic ultrasound using microbubble cavitation to increase muscle perfusion relies on shear-dependent increases in ATP, which can act through a diverse portfolio of purinergic signaling pathways. These events can reverse hindlimb ischemia in mice for >24 hours and increase muscle blood flow in patients with sickle cell disease.URL: http://clinicaltrials.gov. Unique identifier: NCT01566890.
Genetic Susceptibility to Carbamazepine and Allopurinol–Induced Severe Cutaneous Adverse Reactions in Vietnamese
Authors: Dinh Van Nguyen, Hieu Chi Chu, Christopher Vidal, Nguyet Nhu Nguyen, Nga Thi Quynh Do, Tu Thi Linh Tran, Hanh Hong Chu, Ha Thi Thu Nguyen, Huyen Thanh Thuc, Huong Thi Minh Le, Richard B. Fulton, Suran L. Fernando
Journal: Journal of Allergy and Clinical Immunology (2017)
Urinary Tetranor-Pgdm Metabolite from Prostaglandin D2, Is a New Marker to Detect the Severity of Immediate Allergic Reactions Evoked By the Oral Food Challenge in Children with Food Allergy
Authors: Shinichiro Inagaki, Shingo Maeda, Masami Narita, Tatsuro Nakamura, Tatsuo Shimosawa, Takahisa Murata, Yukihiro Ohya
Journal: Journal of Allergy and Clinical Immunology (2017)
Extended follow-up following aggressive resection of locally recurrent rectal cancer.
Authors: Francis Si Wai Zih, Julie I. Hallet, Allison Pang, Bryan J Wells, Calvin Law, Andrew Smith, Carol Jane Swallow
Journal: Journal of Clinical Oncology (2014)

3635 Background: The literature on resection of locally recurrent rectal cancer (LRRC) consists largely of case series based on retrospective data collection with median follow-up of less than 36 months. The goal of the present study was to determine outcomes after 5 to 10 yrs of follow-up in a group of patients managed with a consistent approach. Methods: We identified patients who underwent resection of LRRC between 06/1997 and 05/2005 from the prospective colorectal cancer databases in two cancer centers at the University of Toronto, Canada. Median follow-up time for the entire cohort (n=52) was 44 months; median follow-up in surviving patients (n=25) was 91 months. Results: All 52 patients (median age=60, 31 male) underwent grossly complete resection of their locoregional recurrence; 6 had synchronous distant metastases at the time of LRRC resection. The most common operative procedure for LRRC was a pelvic exenteration with sacrectomy (n=22). 24 patients had received adjuvant XRT for their primary tumor and 26 radionaïve patients received XRT prior to resection for recurrence. At last follow-up, 16 patients were alive with no evidence of disease; 9 alive with disease; 26 had died of disease; and 1 died of other causes. For the entire cohort (n=52), 5-yr and 8-yr Overall Survival (OS) were 49% and 46%, respectively; median OS was 60 months. M1 disease at the time of LRRC resection was associated with inferior OS (p=0.04). Predictors of improved OS included node negative primary cancer (p=0.02); receipt of systemic chemotherapy prior to LRRC resection (p=0.02); and R0 margin of resection on LRRC (p<0.0001). Sacrectomy (n=30) was not a prognostic factor. In patients with no distant metastases at the time of LRRC resection (M0, n=46), 5-yr and 8-yr re-recurrence-free survival were 35% and 31%, respectively. Patients who were clinically M0 at time of LRRC resection in whom R0 resection was achieved (n=36) had 5-yr and 8-yr OS of 65% and 61%, respectively; in that group, re-recurrence-free survival at 5 and 8 yrs were 45% and 39%, respectively. Conclusions: Margin negative resection of LRRC can result in extended survival beyond 5 yrs. Use of preoperative systemic chemotherapy and sacrectomy may be associated with improved results.