Executive Summary

Radiotherapy

Evidence-Based Recommendations

Grade *

Patients with soft tissue sarcoma requiring radiotherapy to be managed through a specialised sarcoma centre to reduce local recurrence and rate of major wound complication.

B

Surgery

Evidence-Based Recommendation

Grade *

Patients with suspected sarcoma to be referred to a specialised sarcoma centre for surgical management to reduce the risk of local recurrence, surgical complication, and to improve limb salvage and survival.

B

Practice Points

Patients with suspected sarcoma should be referred to specialized sarcoma centre early for management including planned biopsy.

Biopsy

Evidence-Based Recommendation

Grade *

Preoperative co-axial core biopsy of suspected retroperitoneal sarcomas is recommended. 

B 

Practice Point

Patients with suspected retroperitoneal sarcoma should be referred to a specialised sarcoma multidisciplinary team for early management including preoperative biopsy. This enables accurate histological and molecular subtyping for multidisciplinary treatment planning.

Radiotherapy

Evidence-Based Recommendation

Grade *

Patients with localised well-differentiated and grade 1-2 dedifferentiated liposarcoma could be considered for pre-operative radiation therapy to improve local/abdominal recurrence free survival. Radiation therapy should not be recommended for other histologic subtypes.

C

Patients with localised retroperitoneal sarcoma should not routinely be recommended radiation therapy with the aim to improve recurrence free survival.

B

Patients with localised retroperitoneal sarcoma should not routinely be recommended radiation therapy with the aim to improve overall survival.

B

Patients with retroperitoneal sarcoma undergoing pre-operative radiation therapy should be informed of the increased risk of serious adverse events in the peri-operative period.

B

Practice Points

Patients with retroperitoneal sarcoma should be referred to specialist sarcoma centre for management.

The rationale and level of evidence for pre-operative radiation therapy for localised liposarcoma should be carefully discussed by the multidisciplinary sarcoma team with the patients.

Multivisceral resection

Evidence-Based Recommendation

Grade *

MVR may be considered for localised resectable retroperitoneal sarcoma with the aim to improve histological margins and may decrease abdominal recurrence.

C

MVR is safe and has comparable perioperative morbidity and mortality outcomes with simple resection.

B

Practice Points

Preoperative radiological assessment by an experienced radiologist as part of the sarcoma multidisciplinary team is also essential in operative planning.

As surgery remains the mainstay of curative therapy for retroperitoneal sarcoma, the oncologic benefit of multivisceral resection should be assessed and balanced against the expected perioperative morbidity in individual patients. 

Chemotherapy

Evidence-Based Recommendation

Grade *

The use of perioperative chemotherapy in primary localised retroperitoneal sarcoma is not the current standard of care.

C

Practice Point

Patients with primary localised retroperitoneal sarcoma should be managed by a specialised sarcoma centre. Patients may be considered for perioperative chemotherapy in a clinical trial setting.

Pelvic Ewing Sarcoma

Evidence-Based Recommendation

Grade *

Delays in surgery (alone or with postoperative radiation therapy) for localised pelvic Ewing sarcoma beyond the recommended timepoint by treatment protocol should be avoided.

C

Practice Point

Patients with pelvic Ewing sarcoma should be managed within a multidisciplinary team who should work closely during the initial chemotherapy period to coordinate the optimal time for disease response evaluation, restaging imaging, and local therapy as per treatment protocol.

Ewing Sarcoma – High Dose Chemotherapy

Evidence-Based Recommendation

Grade *

Selected patients with relapsed Ewing sarcoma could be considered for high dose chemotherapy with autologous stem cell transplant. These patients should be managed in specialised sarcoma centres.

B

Rhabdomyosarcoma – High Dose Chemotherapy

Evidence-Based Recommendation

Grade *

Patients with rhabdomyosarcoma should not undergo high dose chemotherapy outside of a clinical trial setting.

C

Practice Point

Prospective high-quality trials are required to clarify the role of high dose chemotherapy in rhabdomyosarcoma.