Table 1 Summary of consensus statements
From: Seeking international consensus on approaches to primary tumour treatment in Ewing sarcoma
| Patient pathways and services |
| Imaging at presentation should include |
| Conventional X-rays in 2 planes (Strong) |
| MRI of the whole involved compartment and adjacent joints (Strong) |
| Staging CT of chest (Moderate) |
| PET/CT (Moderate) |
| Isotope bone scan (Moderate) |
| Bone marrow sampling (Moderate) |
| Patients should be managed within a properly constituted MDT (Strong) |
| Services should have access to the following |
| Whole body MRI (Strong) |
| Whole body CT/PET (Strong) |
| Specialist surgical teams (Strong) |
| Expert limb fitting/prosthetic services (Strong) |
| Specialist sarcoma rehabilitation (Strong) |
| Clinical nurse specialist support (Strong) |
| Clinical trials (Strong) |
| Radiotherapy by IMRT (Strong) |
| Radiotherapy by proton beam (Strong) |
| Timing and approaches to decisions about local treatment |
| Patients should have the opportunity to explore local treatment options as soon after diagnosis as possible (Strong) |
| Decisions about local therapy should be made in collaboration with patients and families (Strong) |
| It is possible to make a decision about radiotherapy based on the imaging at presentation in some situations (Moderate) |
| The radiological response to chemotherapy is important when considering local therapy options (Strong) |
| With widespread bone metastases, radiotherapy alone to the primary tumour is routinely indicated (Strong) |
| With oligometastases, radiotherapy alone may be considered as well as treatment to the oligometastases (Strong) |
| Patients with pulmonary metastases should be considered for the same local treatment as those without (Strong), including potentially morbid resections (Moderate) |
| Pathology and molecular biology |
| Patients should have biopsies in the bone cancer centre (Strong) |
| Core needle biopsies or open biopsies are preferred (Strong) |
| Specimens should be tested for cytogenetic abnormalities (Strong) |
| Oligometastases in lymph nodes or bone should be biopsied (Moderate) |
| Tissue is banked for research (Strong) |
| Assessment of histological response is important when considering the effectiveness of local treatment (Strong) |
| An adequate response to chemotherapy should be taken as > 90% necrosis (Moderate) |
| Surgical margin status is a reliable indicator of tumour left in the patient (Moderate) |
| An adequate surgical margin is one in which there is no viable tumour at the edge of the resection specimen (Moderate) |
| Surgery |
| The surgical resection should be planned to include the biopsy track (Strong) |
| An adequate surgical margin is one in which all of the anatomical structures involved at presentation are completely removed (Strong) |
| Where feasible it is reasonable to consider resection of peri-lesional oedema (Moderate) |
| The radiological response to neoadjuvant chemotherapy should be considered when planning surgery (Strong) |
| Pelvic spacers may have a role in reducing the morbidity of radiotherapy (Moderate) |
| Radiotherapy has a negative impact on outcomes after endoprosthetic replacement (moderate) |
| Radiotherapy has an negative impact on outcomes after allograft reconstruction (Moderate) |
| Radiotherapy does not make surgery more difficult technically (Moderate) |
| There is no role for debulking surgery when a tumour cannot be completely resected (Strong) |
| Local recurrence has an impact on overall survival (Strong) |
| Anatomical site variations |
| Pelvis and sacrum |
| Tumours which cross the midline in the sacrum are not considered resectable because of the morbidity associated with surgery (Strong) |
| Tumours with major visceral involvement or requiring pelvic organ removal may also be considered too morbid to resect (Moderate) |
| Definitive radiotherapy is indicated for unresectable sacral tumours (Strong) |
| Protons may be advantageous in the sacrum (Strong) |
| Preoperative radiotherapy may be preferred when the tumour volume is large (Moderate) |
| Radiotherapy is likely to be associated with increased complication rates (Strong) |
| Spine |
| Protons may be of some benefit in the spine (Strong) |
| The type of spinal reconstruction can affect the choice of radiotherapy treatment modality (Strong) |
| Patients with a possible Ewing’s tumour of the spine without neurological signs should have a biopsy before decompressive surgery (Strong) |
| Urgent surgery is recommended if there is a Ewing’s tumour of the spine causing neurological compromise (Moderate) |
| Radiotherapy is usually indicated after decompressive surgery (Strong) and should include the original tumour volume and all areas potentially contaminated by surgery (Strong) |
| Chest |
| A pleural effusion in relation to a chest wall tumour is not a definite indication for radiotherapy preoperatively (Moderate) |
| A pleural effusion in relation to a chest wall tumour may be an indication for post operative radiotherapy (Moderate) |
| Pleural involvement with a primary tumour may be an indication for preoperative (None) or postoperative (Moderate) radiotherapy |
| Extremity |
| Amputation is considered less often than for osteosarcoma (Strong) |
| Amputation may be indicated if negative margins cannot otherwise be achieved (Moderate) |
| If resection of a distal leg tumour would lead to inadequate margins or a foot with poor function, below knee amputation is indicated (Strong) |
| Amputation is less often recommended in the upper extremity (Moderate) |
| In the proximal tibia, amputation does not necessarily lead to better outcomes than proximal tibial replacement and radiotherapy (Moderate) |
| Radiotherapy can be added to surgery in the tibia but accepting a high risk of local complications (Moderate), therefore preoperative radiotherapy may be preferred (Moderate) |
| Local therapy in advanced disease |
| Suspected solitary bone metastases should be biopsied at presentation if possible (Strong) |
| Solitary bone metastases may be treated by surgery, radiotherapy or both if the morbidity is acceptable (Strong) |
| If there are widespread bone metastases, radiotherapy is indicated when symptomatic (Strong) |
| Potentially involved lymph nodes should have sampling or biopsy before chemotherapy if possible (Strong) |
| It is appropriate to surgically resect lymph nodes if there is suspicion of tumour involvement (Moderate) |
| It is reasonable to consider radical surgery such as amputation or hemipelvectomy to treat locally recurrent disease if there are no metastases (Strong) |