Suzanne E. J. Kaal

Age as an independent prognostic factor for survival of localised synovial sarcoma patients

Background:We performed a retrospective nationwide study to explore age as a prognostic factor in synovial sarcoma patients.Methods:Data on 613 synovial sarcoma patients were obtained from the Netherlands Cancer Registry. The prognostic relevance of age groups (children, adolescent and young adults (AYAs), adults, and elderly) was estimated by Kaplan–Meier survival curves and multivariable Cox-proportional hazards modelling.Results:A total of 461 patients had localised disease at diagnosis. The 5-year overall survival (OS) was 89.3±4.6%, 73.0±3.8%, 54.7±3.6%, and 43.0±7.0% in children (n=54), AYAs (n=148), adults (n=204), and elderly (n=55), respectively. Treatment modalities had no significant effect on survival in the univariable analysis. Multivariable analysis identified age at diagnosis, tumour localisation, and tumour size as significant factors affecting OS. Both tumour localisation and size were equally distributed over the age groups.Conclusions:We show that outcome of synovial sarcoma patients significantly decreases with age regardless of primary tumour site, size, and treatment.

Advanced soft-tissue sarcoma and treatment options: critical appraisal of trabectedin

Soft-tissue sarcomas (STS) are a heterogeneous group of rare solid tumors of mesenchymal origin. This paper reviews the current status of systemic treatment in advanced and metastatic soft tissue sarcomas, with an emphasis on trabectedin. Trabectedin is a unique type of chemotherapeutic agent with multiple potential mechanisms of action. We discuss the putative mechanisms, as well as the toxicity and administration schedules of trabectedin, followed by its efficacy in first-line systemic therapy and beyond first-line systemic therapy.

Gastrointestinal stromal tumours: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up

Fondazione IRCCS Istituto Nazionale dei Tumori and University of Milan, Milan, Italy; Instituto Portugues de Oncologia de Lisboa Francisco Gentil, EPE, Lisbon, Portugal; University Hospital Essen, Essen Germany; Department of Oncological Orthopedics, Musculoskeletal Tissue Bank, IFO, Regina Elena National Cancer Institute, Rome, Italy; Klinikum Stuttgart-Olgahospital, Stuttgart, Germany; Institut Curie, Paris, France; NORDIX, Athens, Greece; Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; Vienna General Hospital (AKH), Medizinische Universität Wien, Vienna, Austria; Hospital Universitario Virgen del Rocio-CIBERONC, Seville, Spain; Centro di Riferimento Oncologico di Aviano, Aviano; Ospedale Regionale di Treviso ‘S.Maria di Cà Foncello’, Treviso, Italy; Integrated Unit ICO Hospitalet, HUB, Barcelona, Spain; Sarcoma Unit, University College London Hospitals, London, UK; Skane University Hospital-Lund, Lund, Sweden; N. N. Blokhin Russian Cancer Research Center, Moscow, Russian Federation; Institute of Scientific Hospital Care (IRCCS), Regina Elena National Cancer Institute, Rome; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan; Istituto Ortopedico Rizzoli, Bologna; Azienda Ospedaliera Universitaria Careggi Firenze, Florence, Italy; Department of Medical Oncology, Leiden University Medical Centre, Leiden, The Netherlands; Institut Jules Bordet, Brussels, Belgium; Candiolo Cancer Institute, FPO IRCCS, Candiolo, Italy; Department of Radiotherapy, The Netherlands Cancer Institute, Amsterdam and Department of Radiotherapy, Leiden University Medical Centre, Leiden, The Netherlands; Turku University Hospital (Turun Yliopistollinen Keskussairaala), Turlu, Finland; Oxford University Hospitals NHS Foundation Trust, Oxford, UK; Mannheim University Medical Center, Mannheim; Department of Medicine III, University Hospital, Ludwig-Maximilians-University Munich, Munich, Germany; Helsinki University Central Hospital (HUCH), Helsinki, Finland; Royal Marsden Hospital, London; The Institute of Cancer Research, London, UK; University Medical Center Groningen, Groningen; Radboud University Medical Center, Nijmegen, The Netherlands; University Hospital Motol, Prague; Masaryk Memorial Cancer Institute, Brno, Czech Republic; Gustave Roussy Cancer Campus, Villejuif, France; Maria Skłodowska Curie Institute, Oncology Centre, Warsaw, Poland; Tel Aviv Sourasky Medical Center (Ichilov), Tel Aviv, Israel; Medical Oncology, University Hospital of Lausanne, Lausanne, Switzerland; Azienda Ospedaliera, Universitaria, Policlinico S Orsola-Malpighi Università di Bologna, Bologna; Azienda Ospedaliero, Universitaria Cita della Salute e della Scienza di Torino, Turin, Italy; Fundacio de Gestio Sanitaria de L’hospital de la Santa Creu I Sant Pau, Barcelona, Spain; Helios Klinikum Berlin Buch, Berlin, Germany; YCRC Department of Clinical Oncology, Weston Park Hospital NHS Trust, Sheffield, UK; Aarhus University Hospital, Aarhus, Finland; Leuven Cancer Institute, Leuven, Belgium; Department of Medical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands; Fondazione Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Nazionale dei Tumori, Milan, Italy; Department of Oncology, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway; Institute of Oncology of Ljubljana, Ljubljana, Slovenia; Netherlands Cancer Institute Antoni van Leeuwenhoek, Amsterdam, The Netherlands; University College Hospital, London, UK; Gerhard-Domagk-Institut für Pathologie, Universitätsklinikum Münster, Münster, Germany; Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway; Centre Leon Bernard and UCBL1, Lyon, France

Adolescent and young adult (AYA) lymphoma survivors report lower health-related quality of life compared to a normative population: results from the PROFILES registry

Abstract Background: Trying to simultaneously achieve developmental milestones and cope with a life-threatening disease may place adolescents and young adults (AYAs) at risk for impaired health-related quality of life (HRQoL) later in life. The aim of this study was to examine differences in HRQoL between AYA lymphoma survivors and a normative population and to determine sociodemographic, clinical and long-term symptom-related factors associated with HRQoL. Material and methods: This study was part of a longitudinal, population-based survey among lymphoma survivors diagnosed between 1999 and 2012. The AYA survivor sample (18–39 years at time diagnosis) was compared to a sex- and age-matched normative population on HRQoL (EORTC-QLQ-C30) and psychological distress (HADS). Multiple linear regression analyses were conducted to determine factors associated with HRQoL among survivors. Results: One hundred and ninety-eight AYA lymphoma survivors (58%) responded to the study invitation. Compared to an age- and sex-matched normative population (N = 380), significantly and clinically relevant poorer HRQoL was observed for AYA lymphoma survivors in seven specific domains of HRQoL: physical, role, cognitive, emotional, social functioning, fatigue and financial difficulties (all p < 0.05). In addition, AYA lymphoma survivors less often had a spouse/partner and more often had a lower educational level compared to the normative population. Linear regression analyses showed that being unemployed, female gender, having one or more comorbid conditions, high levels of fatigue and psychological distress were most strongly associated with HRQoL. Conclusions: These findings identify specific domains of life in which cancer has a significant and long-term impact for AYA lymphoma survivors. Future investigations are needed to identify and test administrations and timing of psychosocial support interventions having potential to reduce long-term late effects in specific HRQoL domains and promote function and adaptability after cancer treatment.

Abscopal effect of radiotherapy in a patient with metastatic diffuse-type giant cell tumor

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Empowerment in adolescents and young adults with cancer: Relationship with health‐related quality of life

The difficulties adolescents and young adults (AYAs) encounter during a cancer experience may result in a reduction in or absence of empowerment. The aims of the current study were to assess levels of empowerment and associated (demographic, clinical, or psychological) factors and examine the association between empowerment and health‐related quality of life (HRQOL) among AYA patients with cancer.

Fatal heart failure in a young adult female sarcoma patient treated with pazopanib

Soft tissue sarcomas are rare malignancies of mesenchymal origin. Pazopanib is an oral tyrosine kinase inhibitor, targeting vascular endothelial growth factor receptors (VEGF1-2-3), platelet-derived growth factor receptors (PDGFR-a and -b) and stem cell factor receptor (c-KIT) and is approved for patients with metastatic non-adipocytic soft tissue sarcoma after anthracycline-based chemotherapy [1]. Pazopanib is usually well tolerated, with fatigue, diarrhea, nausea, weight loss and hypertension as the most frequently described side effects [1]. Symptomatic left ventricular systolic dysfunction is a rare side effect, occurring in 1% of patients with soft tissue sarcoma, and fatal outcome has been described once [2]. In this case report, we describe fatal congestive heart failure and hepatotoxicity in a young patient, occurring four weeks after commencing pazopanib, with high serum drug levels of pazopanib.

Experiences of Parents and General Practitioners with End-of-Life Care in Adolescents and Young Adults with Cancer

This study aims to analyze the experiences of Dutch bereaved parents and general practitioners (GPs) with palliative care of AYAs (18-35 years) in the terminal stage. Fifteen parents and nine GPs involved with nine deceased AYAs filled out questionnaires and were interviewed by telephone, respectively. In general, the parents were satisfied with the emotional care they themselves received and the medical care that their child received. The GPs were very satisfied with the cooperation with the palliative team. Gaps are present in the areas of symptom control, communication between hospital professionals and parents, aftercare, and transition between hospital and GP.

The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column

BACKGROUND The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whether chemotherapy is preferred over decompressive laminectomy. OBJECTIVE To underline, in this case series, the efficiency of initial chemotherapy before upfront surgery in the setting of high-grade spinal cord or cauda equina compression of primary Ewing sarcoma. METHODS Fifteen patients with Ewing sarcoma primarily located in the spine were treated at our institution between 1983 and 2015. Localization, neurological deficit expressed as Frankel grade, and outcome expressed as Rankin scale before and after initial chemotherapy, the recurrence rate, and overall survival were evaluated. The multidisciplinary approach of 1 case will be discussed in detail. RESULTS Nine patients (60%) were female. The age at presentation was 15.0 ± 5.5 years (range: 0.9-22.8 years). Ten patients (67%) were initially treated with chemotherapy, and 1 patient (7%) was treated primarily with radiotherapy followed by chemotherapy. The remaining 4 patients (27%) were initially treated with decompressive surgery. All patients treated primarily nonsurgically improved neurologically at follow-up, showing the importance of chemotherapy as an effective initial treatment option. CONCLUSION Adequate and quick decompression of neural structures with similar results can be achieved by chemotherapy and radiotherapy, avoiding the local spill of malignant cells.

The Dutch AYA Outpatient Clinic: Support and Counselling During and After Cancer Treatment

This chapter describes how a start was made with improving age-specific care for Adolescents and Young Adults (AYAs) in the Netherlands. AYA care is delivered to patients diagnosed with cancer between the ages of 18 and 35 years. In developing sustainable improvement in the quality and quantity of life for young adults with cancer, patient participation of young adults was sought from the very beginning. One of the major achievements in the development of AYA care is the establishment of nurse-led AYA Outpatient Clinics. An important part in describing the design and organization of an AYA Outpatient Clinic is the role played by the various clinical nurse specialists involved in AYA care. A case study is used to contextualize AYA care in daily practice.