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.

Screening with a validated screening tool (for example, the National Comprehensive Cancer Network Distress Thermometer and Problem Checklist), assessment and referral to appropriate health professionals or organisations is required to meet the identified needs of an individual, their carer and family.

In addition to the other common issues outlined in the Appendix, specific needs that may arise at this time include the following.

Physical needs

• Decline in functional status (particularly as a result of limb reconstruction or amputation) may affect the patient’s mobility and ability to take part in everyday activities. Referral to an occupational therapist, orthotist/prosthetist and a physiotherapist or exercise physiologist for assessment, education, intervention and compensatory strategies may assist with maintaining mobility. Patients may require prolonged periods of rehabilitation.
• Healing of underlying structures, infection and other complication risks relating to skeletal implants may require input from wound nurse specialists and infection control specialists.
• Patients who have had a limb amputated to treat their sarcoma require rapid and easy access to prosthetic services.
• Treatment for other physical symptoms such as pain, fatigue and musculoskeletal dysfunction may be required.
• Patients will require ongoing nutritional screening, assessment and management. Reduced oral intake and/or swallowing difficulties and weight loss require referral to a dietitian and speech pathologist (for swallowing difficulties).
• Assistance with managing complex medication regimens, multiple medications, assessing side effects and assistance with difficulties swallowing medications may be required. Refer to a pharmacist if necessary.

Psychological needs

• Disfigurement and scarring from appearance-altering treatment and the need for a prosthesis may require referral to a specialist psychologist, psychiatrist, orthotist/prosthetist or social worker.
• Patients may need support with emotional and psychological issues including, but not limited to, body image concerns, fatigue, existential anxiety, treatment phobias, anxiety/depression, psychosexual issues such as potential loss of fertility, interpersonal problems and sexuality concerns.

Social/practical needs

• Ensure the patient attends appointments.
• Patients may experience isolation from their normal support networks, particularly for rural patients who are staying away from home for treatment.
• Financial issues related to loss of income and additional expenses as a result of illness and/or treatment may require support.
• Help with legal issues may be required including for advance care planning, appointing a power of attorney, completing a will and making an insurance, superannuation or social security claim on the basis of terminal illness or permanent disability.

Information needs

• Provide appropriate information for people from culturally and linguistically diverse backgrounds.

Spiritual needs

• Multidisciplinary teams should have access to suitably qualified, authorised and appointed spiritual caregivers who can act as a resource for patients, carers and staff.
• Patients with cancer and their families should have access to spiritual support that is appropriate to their needs throughout the cancer journey.

The lead clinician should:

• offer advice to patients and carers on the benefits of or how to access support from sarcoma peer support groups, groups for carers and special interest groups
• discuss the treatment plan with the patient and carer, including the intent of treatment and expected outcomes
• provide the patient and carer with information on possible side effects of treatment, self-management strategies and emergency contacts
• recognise the ability of the patient and carers to comprehend the communication
• initiate a discussion regarding advance care planning with the patient and carer.

The lead clinician should:

• communicate with the person’s general practitioner about their role in symptom management, psychosocial care and referral to local services
• ensure regular and timely two-way communication regarding
  • the treatment plan, including intent and potential side effects
  • supportive and palliative care requirements
  • the patient’s prognosis and their understanding of this
  • enrolment in research and/or clinical trials
  • changes in treatment or medications
  • recommendations from the multidisciplinary team.