STEP 6: Managing recurrent, residual or metastatic disease

Step 6 is concerned with managing recurrent, residual or metastatic disease.

Between 30 and 40 per cent of all patients with sarcomas develop local or distant recurrence (Cancer Council Australia Sarcoma Guidelines Working Party 2014). The risk of recurrence is greatest in the first few years, with approximately two out of three recurrences developing within two years and 95 per cent by five years (of initial diagnosis). Diagnoses can be stratified into risk groups based on the prognostic features of the primary tumour (Cancer Council Australia Sarcoma Guidelines Working Party 2014).

In those patients with recurrent or residual disease there should be timely referral to the original multidisciplinary team (where possible) and thereby quick access to specialist input into care.

The pathway for and manageing patients with recurrent or metastatic sarcoma is a continuum of care within the multidisciplinary team and recapitulates section 4.

Treatment will depend on the location and extent of disease, timing of recurrence, previous management and the patient’s preferences.

Participation in research and/or clinical trials should be encouraged where available and appropriate.

The standard approach to local recurrence parallels the approach to primary local disease, although more often resort to preoperative or postoperative radiation therapy and/or chemotherapy, if not previously carried out.

In the case of isolated lung metastases, treatment is primarily surgical where complete removal of all metastases should be attempted (ESMO 2014a).

Palliative radiation therapy or chemotherapy may be considered for inoperable metastases (ESMO 2014a).

A number of targeted therapies have shown promising results in patients with certain histological types of advanced or metastatic soft tissue sarcoma (NCCN 2016a). The role of chemotherapy for recurrent Ewing’s sarcoma and osteosarcoma should follow protocolised treatment following multidisciplinary team discussion.

The lead clinician should ensure patients receive timely and appropriate referral to palliative care services. Referral should be based on need rather than prognosis.

  • Patients may be referred to palliative care at initial diagnosis.
  • Patients should be referred to palliative care at the first recurrence or progression.
  • Carer needs may prompt referral.

Early referral to palliative care can improve the quality of life for people with cancer (Haines 2011; Temel et al. 2010; Zimmermann et al. 2014). This is particularly true for poor-prognosis cancers (Temel et al. 2010). Furthermore, palliative care has been associated with the improved wellbeing of carers (Higginson & Evans 2010; Hudson et al. 2015).

Ensure carers and families receive information, support and guidance regarding their role according to their needs and wishes (Palliative Care Australia 2005).

The patient and carer should be encouraged to develop an advance care plan (AHMAC 2011). For more information refer patients and carers to Palliative Care Australia.

Participation in research and/or clinical trials should be encouraged where available and appropriate.

  • Australasian Sarcoma Study Group is a national cooperative cancer clinical research group. It coordinates large-scale multi-centred sarcoma trials.
  • Australian Cancer Trials is a national clinical trials database. It provides information on the latest clinical trials in cancer care, including trials that are recruiting new participants. For more information visit the Australian Cancer Trials website.

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

In addition to the common issues outlined in the Appendix, specific issues that may arise include the following.

Physical needs

  • Patients may require treatment for ongoing/new physical symptoms such as pain, fatigue and musculoskeletal dysfunction.
  • Patients 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

  • Emotional and psychological distress may result from fear of death, complications of chemotherapy, existential concerns, anticipatory grief, communicating wishes to loved ones, 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 may result from disease recurrence (patients may need early access to superannuation and insurance).
  • 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.

Rehabilitation may be required at any point of the care pathway, from preparing for treatment through to disease-free survival and palliative care. Issues that may need to be addressed include managing cancer-related fatigue, cognitive changes, improving physical endurance, achieving independence in daily tasks, returning to work and ongoing adjustment to disease and its sequelae.

The lead clinician should ensure there is adequate discussion with the patient and carer about the diagnosis and recommended treatment, including the intent of treatment and its possible outcomes, likely adverse effects and supportive care options available for both the patient and their family/carer.