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CLINICAL OUTCOME IN PATIENTS WITH ORBITAL RHABDOMYOSARCOMA- UPDATED RESULTS FROM A REGIONAL CANCER CENTRE IN NORTH INDIA
Abstract
Background and Aims
Orbital Rhabdomyosarcoma(RMS) is a favourable prognostic tumour with cure rates of more than 90% when treated with combination chemotherapy and local radiotherapy in trial setting.
Methods
The treatment plan for patients diagnosed with orbital RMS was induction chemotherapy (VAC regimen-Vincristine,Actinomycin D,Cyclophosphamide) for 3 courses followed by orbital radiotherapy (45Gray/25fractions/5weeks) followed by further chemotherapy (VAC regimen) up to a total of 12 courses. Clinical data was collected by retrospective chart review from 2010-19. Overall survival (OS) and progression free survival (PFS) were analyzed by Kaplan-Meier method.
Results
Thirty three patients met the study criterion (male:female=18:15). Median age at presentation was 8 years. Orbital biopsy revealed embryonal and alveolar RMS in 29(87.9%) and 4(12.1%) patients respectively. After staging work-up, 4(12.1%),26(78.8%) and 3(9.1%) patients had IRS clinical group II, III and IV disease respectively. Systemic chemotherapy(VAC) was administered in all patients (median-8 courses). Orbital radiotherapy was given in 26(78.8%) patients(median dose 45 Gray;25-curative;1-palliative). After a mean follow-up of 26.7 months (median-17.8 months), 13(39.4%) patients had disease progression(local-10,regional-2,distant-2). Two patients could be successfully salvaged with chemotherapy(ICE regimen-Ifosfamide,Carboplatin,Etoposide) and orbital exenteration. At last follow-up, 9(27.3%) patients had died- 4 due to progressive disease and 5 due to chemotherapy related toxicity. The median PFS was 22.6 months and the median OS had not been reached. The actuarial rates of PFS and OS were respectively 49.2% and 73%(at 2 years) and 49.2% and 66.9%(at 3 years). On univariate analysis, use of chemotherapy(≥8 cycles) and orbital radiotherapy led to significantly enhanced PFS (HR-0.22 and 0.15, P-0.02 and 0.02 respectively). These 2 factors retained prognostic significance on multivariate analysis of PFS (P=0.03 each).
Conclusions
Combination chemotherapy (VAC) and orbital radiotherapy (45Gy) led to modest clinical outcome in patients with orbital RMS at our centre possibly due to high incidence of alveolar RMS(12.1%), metastatic disease(9.1%), poor treatment compliance(18.2%) and toxic death(15.2%).
QUARTET: A SIOP EUROPE PROJECT FOR QUALITY AND EXCELLENCE IN RADIOTHERAPY AND IMAGING FOR CHILDREN AND ADOLESCENTS WITH CANCER
Abstract
Background and Aims
The Quality and Excellence in Radiotherapy and Imaging for Children and Adolescents with Cancer across Europe in Clinical Trials (QUARTET) project is part of the European Society for Paediatric Oncology (SIOPE) Strategic Plan to improve treatment effectiveness and quality of survivorship for children and adolescents with cancer. Through a collaboration with the European Organisation for Research and Treatment of Cancer (EORTC), QUARTET facilitates the review of radiotherapy treatment plans and the analysis of imaging for patients enrolled in clinical trials. The QUARTET project aims to improve the quality of radiotherapy and imaging within clinical trials, support the validity of trial results, and inform future best practice in paediatric radiotherapy and imaging.
Methods
Collaborating with SIOPE study groups and clinical trial sponsors, QUARTET has prepared for delivery of customised quality assurance programmes for several paediatric trials. This has included organisation of the processes for data submission, review, and feedback to investigators. General paediatric and trial-specific Radiotherapy Quality Assurance (RTQA) and imaging guidelines have been developed and incorporate instructions for delineation of target volumes and organs at risk; imaging and treatment planning/delivery requirements; and dosimetric objectives.
Results
Prospective RTQA using QUARTET is approved for six trials encompassing: rhabdomyosarcoma (FaR-RMS), neuroblastoma (LINES, HR-NBL2/SIOPEN, VERITAS), and medulloblastoma (PNET-5, HRMB). One retrospective RTQA analysis for HR-NBL1/SIOPEN is underway. Four imaging studies have been approved across neuroblastoma and rhabdomyosarcoma. QA of molecular radiotherapy dosimetry will be carried out for the VERITAS trial.
Conclusions
Introducing comprehensive radiotherapy guidelines and centralised prospective quality assurance for paediatric clinical trials has the potential to improve the quality of radiotherapy in clinical trials and reduce the disparities in outcomes we see across Europe. Feedback via conferences, publications, the SIOPE Radiation Oncology Working Group, and disease-specific study groups will therefore be essential. Dissemination of findings will re-inforce and improve standards of care.
A SURVEY OF ONCOLOGY SERVICES IN SUB SAHARAN AFRICA - A WINDOW INTO PAEDIATRIC CANCER CARE
Abstract
Background and Aims
Tremendous progress has been made in paediatric oncology training in Sub Saharan Africa (SSA). Despite these positive strides, survival outcomes remain abysmal compared to high income countries. To improve outcomes, dedicated paediatric oncology centres with facilities for diagnosis, imaging, chemotherapy, radiotherapy and rehabilitation are needed to provide comprehensive care. The purpose of this study is to assess the current state of oncology care in SSA which is a direct reflection of the state of paediatric oncology care.
Methods
An anonymous online survey of cancer management in SSA Countries was conducted among African Organisation for Research and Training in Cancer (AORTIC) members in February 2019 using a structured questionnaire in English, French and Portuguese.
Results
Thirty-seven respondents from 23 Sub Saharan African countries of whom 91% were oncologist participated in the survey. Eighteen (60%) institutions had radiotherapy equipment made up of 14 Linacs and 8 Cobalt 60 machines. Seven countries had no radiotherapy access. Radiotherapy delivery was via 2D and 3D conformal technique and IMRT was practiced in 4 centres. Average waiting time to commence radiotherapy is 8 weeks but as high as 12 weeks in some (23%) institutions. Chemotherapy agents for pediatric cancers were available in almost all centres ( 90%). Majority (80%) had X-ray, USG and CT Scans machinesin their facilitiesn. MRI, Bone Scan and Pet Scans were however available in only 42.%, 16% and 5% respectively. The average turnaround time for pathology services available in 80% of institutions was 1-3 weeks. Over half (56%) performed IHC studies. Out of pocket payment was required in 60% of cases and late presentation was the most significant factor affecting treatment outcome
Conclusions
The challenge of late presentation, inadequate radiotherapy services and lack of political will power to support paediatric cancer treatment continues to be a severe handicap requiring urgent intervention.
HYPOFRACTIONATED ACCELERATED RADIOTHERAPY FOR PEDIATRIC OSTEOSARCOMA
Abstract
Background and Aims
Radiotherapy could worthily contribute to local control of osteosarcoma when surgery is unfeasible or incomplete and for symptoms palliation. An unconventional intensive hypofractionated accelerated regimen (Hypo-ART) has been adopted at our Institution, in the attempt to overcome the theoretical radio-resistance of osteosarcoma. To evaluate the effectiveness of this strategy, we retrospectively reviewed outcomes and treatment-related toxicities in a predominantly pediatric and AYA series.
Methods
Between 1992 and 2019, 48 patients (median age 15 years, 8-46; 83% <21 years), received Hypo-ART (3 Gy bid, total dose 42-48 Gy, 7-8 treatment days) to 64 sites. Clinical response was evaluated with respect to symptoms relief, functional improvement and objective tumor volume shrinkage. Local control was defined as diagnostic imaging-based durable stabilization/regression and/or persistent clinical response. Acute and late toxicity were reported according to CTCAEv5.0.
Results
Radiotherapy indication was curative for 37 sites and palliative for 27. Twenty-six sites were treated at diagnosis (24 primary sites) and 38 at relapse (30 metastatic sites). With a median F-UP of 89 months (IQR: 62-210), only 11 sites (17%) developed local failure. Pain reduction was observed in 37/39 (95%) symptomatic sites and analgesics were lowered/interrupted in 20/30 (67%) courses. Functional improvement and tumor shrinkage were documented for 48/49 and 34/38 assessable sites, respectively. Nine/64 (14%) and 9/37 (24%) irradiated sites experienced grade 3-4 reversible acute or late toxicity, respectively. The 5-year local PFS resulted significantly different between sites receiving curative and palliative treatment (79% vs 25%, p: 0.0029), primary and metastatic sites (80% vs 50%, p: 0.0028) and sites treated at diagnosis and at recurrence (82% vs 55%, p: 0.0089).
Conclusions
Hypo-ART proved to be a feasible, effective and fast treatment for osteosarcoma, with a local control rate of 83%. Greater efforts are needed to improve local control in higher risk subgroups for which carbon ion radiotherapy could be promising.