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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)