4.2 Treatment options
The advantages and disadvantages of each treatment and associated potential side effects should be discussed with the patient and their carer/family.
Surgery is the most common treatment option for most types of sarcoma. This consists of resection and reconstruction.
The objective of surgical resection is to achieve adequate oncologic margins. Decisions about the optimal surgical procedure are made with reference to the tumour type, extent and response to neoadjuvant therapy if appropriate (refer to sections 4.2.2 and 4.2.3).
The objective of reconstruction is to promote wound healing, optimise function and improve the appearance of the affected area.
When surgery involves the limb, the preference is for limb salvage surgery, though occasionally ablative surgery (amputation) may be required.
Most patients are considered as candidates for limb salvage surgery. When considering the feasibility of limb preservation, the following should be taken into account:
- the outcome of surgery in regard to local recurrence
- distant metastasis and survival outcome (this should be comparable to that of ablative surgery)
- risk of complications
- possible re-operations and secondary amputation
- the functional outcome (this should be equivalent to or better than amputation).
All surgical options (including amputation) should be discussed with and acceptable to the patient.
It is important that the rehabilitation team has specific skills with limb salvage surgery and amputee rehabilitation. Upper limb amputees should receive rehabilitation as soon as possible after surgery.
Appropriate vascular and plastic surgical reconstructive options should be available.
Training, experience and treatment centre characteristics
The training and experience required of the surgeon is as follows:
- surgeon (FRACS or equivalent) with adequate training and experience and institutional cross- credentialling and agreed scope of practice within this area (ACSQHC 2004)
- adequate training including subspecialty training at a national or international centre of excellence with continued practice as part of a recognised multidisciplinary team
- plastic surgeon with an interest and expertise in sarcoma reconstructive surgery.
Hospital or treatment unit characteristics for providing safe and quality care include:
- appropriate nursing and theatre resources to manage complex surgery
- theatre with prosthetics capability
- 24-hour medical staff availability
- 24-hour operating room access
- specialist pathology expertise / molecular pathology
- full anatomic imaging modalities
- specialist interventional diagnostic radiology and nuclear medicine expertise.
Surgical volumes
High-volume centres generally have better clinical outcomes (Bhangu et al. 2004; Gutierrez et al. 2007; Sampo et al. 2012; Stiller et al. 2006) and are associated with improved rates of functional limb preservation, lower rates of local recurrence, good rates of overall survival and improved quality of life
(Cancer Council Australia Sarcoma Guidelines Working Party 2014). Centres that do not have sufficient caseloads should establish processes to routinely refer surgical cases to a high-volume centre.
All patients with large, localised, soft tissue tumours should be considered for radiation therapy by a radiation oncologist with experience in treating sarcomas and involvement in multidisciplinary care. For smaller tumors under 5 cm and lower grade tumours in more difficult anatomic sites, consideration should still be given to radiation therapy, given the implications of local recurrence in these anatomic sites (Pisters et al. 2016).
For soft tissue sarcoma, radiation therapy (external beam, brachytherapy, intensity-modulated radiation therapy, particle beam) must be considered before or after surgery.
The timing of radiation therapy needs to be individualised dependent upon resection and reconstructive considerations.
In general, radiation therapy for bone sarcomas is mainly used for palliation (ESMO 2014a). In Ewing’s sarcoma, radiation therapy may be considered as part of the treatment protocol.
Training, experience and treatment centre characteristics
Training and experience required of the appropriate specialist(s):
- radiation oncologist (Fellowship of the Royal Australian and New Zealand College of Radiologists or equivalent) with adequate training and experience with institutional credentialling and agreed scope of practice in sarcoma (ACSQHC 2004)
- adequate training including subspecialty training at a national or international centre of excellence with continued practice as part of a recognised multidisciplinary team.
Radiation oncology centre characteristics for providing safe and quality care include:
- radiation therapists and medical physicists with experience in sarcoma care
- access to radiation therapy nurses, allied health professionals (especially for nutrition health and advice) occupational therapists and psychological support
- access to CT and MRI scanning for simulation and planning.
All patients with osteosarcoma and Ewing’s sarcoma should be considered for protocolised pre- and/or postoperative chemotherapy by a medical oncologist with experience in treating sarcoma and involvement in multidisciplinary care (ESMO 2014a). Other forms of bone sarcomas should be treated as per multidisciplinary team discussion.
Rhabdomyosarcoma should be treated with protocolised pre- and/or postoperative chemotherapy by a medical oncologist with experience in treating sarcoma and involvement in multidisciplinary care.
For patients with other forms of localised soft tissue sarcoma, chemotherapy is not the current standard of care, and patients should be treated as per the multidisciplinary team’s treatment plan.
Training, experience and treatment centre characteristics
The following training and experience is required of the appropriate specialist(s):
- Medical oncologists (Fellowship of the Royal Australasian College of Physicians or equivalent) must have adequate training and experience with institutional credentialling and agreed scope of practice within this area (ACSQHC 2004).
- Adequate training must include subspecialty training at a national or international centre of excellence with continued practice as part of a recognised multidisciplinary team.
- Nurses must have specialist training in chemotherapy handling, administration and disposal of cytotoxic waste and advanced understanding of chemotherapy toxicities. They must also have advanced central venous access knowledge and skills.
- Chemotherapy should be prepared by a pharmacist with adequate training in chemotherapy medication, including dosing calculations according to protocols, formulations and/or preparation.
- In a setting where no medical oncologist is locally available, some components of less complex therapies may be delivered by a medical practitioner and/or nurse with training and experience and with credentialling and agreed scope of practice within this area under the guidance of a medical oncologist. This should be in accordance with a detailed treatment plan or agreed protocol and with communication as agreed with the medical oncologist or as clinically required.
Hospital or treatment unit characteristics for providing safe and quality care include:
- a clearly defined path to emergency care and advice after hours
- access to basic haematology and biochemistry testing
- cytotoxic drugs prepared in a pharmacy with appropriate facilities
- occupational health and safety guidelines regarding the handling of cytotoxic drugs, including safe prescribing, preparation, dispensing, supplying, administering, storing, manufacturing, compounding and monitoring the effects of medicines (ACSQHC 2011)
- guidelines and protocols regarding delivery treatment safely (including dealing with extravasation of drugs)
- mechanisms for coordinating combined therapy (chemotherapy and radiation therapy), especially where facilities are not collocated.
Timeframes should be informed by evidence-based guidelines (where they exist) while recognising that shorter timelines for appropriate consultations and treatment can reduce patient distress.
The following recommended timeframes are based on expert advice from the Sarcoma Working Group:3
- Patients must begin their treatment within three weeks of the decision to treat.
3 The multidisciplinary experts who participated in a clinical workshop to develop content for the sarcoma optimal care pathway are listed in the acknowledgements.