Guidelines for the Management of Soft Tissue Sarcomas

Abstract

These guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group and are intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. The guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) were used as the basis for discussion and adapted according to UK clinical practice and local requirements. Note was also taken of the National Institute for Health and Clinical Excellence (NICE) improving outcomes guidance (IOG) for people with sarcoma and existing technology appraisals. The guidelines are not intended to challenge NICE guidance but discrepancies may exist where current guidance does not reflect an international standard of care owing to the ever-evolving nature of cancer treatment. It is acknowledged that these guidelines will require updating on a regular basis. An appendix lists the key recommendations which are summarised below. Any patient with a suspected soft tissue sarcoma should be referred to a diagnostic centre and managed by a specialist sarcoma multidisciplinary team. Surgical excision followed by post operative radiotherapy is the standard management of high grade limb sarcomas although occasionally amputation remains the only option. Pre-operative treatment with chemotherapy or radiotherapy should be considered for patients with borderline resectable tumours. Isolated limb perfusion may permit limb salvage in some cases where amputation is the only other option. Adjuvant chemotherapy is not routinely recommended but may be considered in certain specific situations. Regular follow up is recommended to assess local control and the development of metastatic disease. Single agent doxorubicin is the standard first line therapy for metastatic disease. Ifosfamide is an alternative if anthracyclines are contraindicated. Combination therapy may be considered in individual patients. Second line agents include ifosfamide, dacarbazine, trabectedin and the combination of gemcitabine + docetaxel. Surgical resection of local recurrence and pulmonary metastases should be considered in individual patients. There is specific guidance on the management of retroperitoneal and uterine sarcomas.

Putative DNA/RNA helicase Schlafen-11 (SLFN11) sensitizes cancer cells to DNA-damaging agents

“…21 and the National Comprehensive Cancer Network guidelines for soft tissue sarcoma (http://www.nccn.org)]. It must be noted, however, that these drugs show activity in a minority of sarcomas, so other molecular determinants must explain why response rates for most subtypes of sarcomas remain extremely low.…”

Case Report: Myxoid Liposarcoma in Retroperitoneum

“…Preoperative radiotherapy is advantageous for radiosensitive subtypes in which tumor shrinkage allows complete surgical resection [11]. The myxoid liposarcoma is a radiosensitive type, in the case reported, despite the large size of the tumor it was possible to obtain margin with the block resection surgery, for the reason of the non-indication of preoperative radiotherapy.…”

“…A biopsy is not necessary before surgery, except in cases where neoplasms other than sarcomas are suspected. Biopsy is indicated for patients who are candidates for chemotherapy and/or preoperative radiotherapy, and Image-guided core needle biopsy is preferred [11].…”

Acral manifestations of soft tissue tumors

“…Wide local excision (most would agree that wide local excision entails an intact fascial layer or 1 cm of normal surrounding tissue 55 ) followed by close observation is the minimum treatment for these tumors. More aggressive care is necessary for epithelioid and clear cell sarcomas given that more than half of patients die within 10 years of these diagnoses, usually after a relentlessly progressive series of recurrences.…”

“…Extended surveillance is indicated, because recurrences can appear after decades of quiescence. 55,[57][58][59][60] Acral myxoinflammatory fibroblastic sarcoma…”