Recommendations

Strength Of recommendation

1-Work up for newly diagnosed Ewing Sarcoma

Image guided biopsy with IHC is recommended.

Strong recommendation

Molecular studies are recommended as needed guided by expert opinion.

Conditional recommendation

Contrast enhanced MRI of the primary site is recommended.

Strong recommendation

We recommend PET/CT if available or CT chest and bone scan if PET/CT is unavailable.

Strong recommendation

Bone marrow biopsy is recommended if PET/CT is unavailable or positive uptake of bone marrow in PET/CT.

Strong recommendation

2- First line therapy for non-metastatic primary tumour

(neoadjuvant/adjuvant)

Multiagent chemotherapy for at least 9 weeks prior to local therapy is recommended (interval compressed chemotherapy).

All patients are recommended to continue adjuvant chemotherapy after local control till 28 weeks.

Strong recommendation

Strong recommendation

Preferred Regimen

VDC/IE (Vincristine, doxorubicin and cyclophosphamide) alternating with (Ifosfamide and etoposide) every 2 weeks with GCSF for a total of 14 cycles.

Strong recommendation

Restage after neoadjuvant therapy before local control

CT chest and contrast enhanced MRI of primary site are recommended.

Strong recommendation

Local Control Therapy for stable/improved disease following neoadjuvant therapy

We recommend wide surgical excision and adjuvant chemotherapy. Radiotherapy is recommended if positive surgical margins.

Strong recommendation

Definitive radiotherapy and adjuvant chemotherapy are recommended for irresectable tumours.

Strong recommendation

3- First line therapy for metastatic disease at initial presentation

Multiagent chemotherapy for at least 9 weeks prior to local therapy is recommended (interval compressed chemotherapy).

Preferred Regimen

VDC/IE (Vincristine, doxorubicin and cyclophosphamide) alternating with (Ifosfamide and etoposide) every 2 weeks with GCSF for a total of 14 cycles.

All patients are recommended to continue adjuvant chemotherapy after local control till 28 weeks.

Strong recommendation

Local control for metastatic disease

We recommend wide surgical excision and adjuvant chemotherapy. Radiotherapy is recommended if positive surgical margins.

Strong recommendation

Definitive radiotherapy and adjuvant chemotherapy are recommended for irresectable tumours.

Strong recommendation

Management of metastases

For lung only metastases with partial response to neoadjuvant treatment, resection and whole lung irradiation are recommended.

Strong recommendation

For lung only metastases with complete response to neoadjuvant treatment, whole lung irradiation is recommended.

Strong recommendation

For bone metastases it is recommended to give radiotherapy to metastatic sites.

Strong recommendation

4- Radiotherapy

Timing of RT

For patients receiving radiation therapy only it is recommended to be delivered at the beginning of week 13 concurrently with chemotherapy.

Strong recommendation

If post-operative radiotherapy is recommended, consider starting at week 15 concurrently with chemotherapy starting on day 1 of the cycle as soon as possible after surgery.

Strong recommendation

Patients with recent cord compression are recommended to start emergency concurrent radiotherapy and chemotherapy starting from day 1 first cycle.

Strong recommendation

Concurrent chemotherapeutic agents

Ifosfamide, etoposide, cyclophosphamide and vincristine should be given with radiotherapy. It is recommended to withhold doxorubicin with radiotherapy and re-institute after completion of radiation.

Strong recommendation

5- Treatment of recurrent/relapsed Ewing Sarcoma

Chemotherapy

Recommended chemotherapy combination

·       Irinotecan and temozolomide in 21-day interval cycles, Or

·       Ifosfamide, carboplatin and etoposide (if > 6 months).

Strong recommendation

Surgery

Surgical resection of both local and metastatic sites (especially pulmonary) if feasible is recommended.

Strong recommendation

Radiotherapy

Radiation is recommended either definitive or postoperative.

Strong recommendation

6- Surveillance – Follow up - for Ewing Sarcoma patients

X-ray of the primary site is recommended every 4 months for the first 2 years and as clinically warranted.

Strong recommendation

CT chest every 4 months is the recommended chest imaging in the first 2 years. Chest X-ray is recommended for chest imaging in later years.

Strong recommendation

It is recommended to increase intervals of imaging of primary site and chest after 24 months and annually after 5 years (indefinitely).

Strong recommendation