The causes of most NETs are not fully understood, and there is currently no clear prevention strategy.
However, when there is a history of hereditary conditions the risk is greater, and genetic screening and regular reviews need to be in place to detect early asymptomatic cancer (refer to section 1.3).

The risk factors for developing NETs include (Cancer Centre 2021; Roswell Park 2021; Department of Health and Ageing 2013):

• hereditary conditions such as multiple endocrine neoplasia type 1 (MEN-1), multiple endocrine neoplasia type 2 (MEN-2), von Hippel-Lindau disease (VHL), phacomatoses (neurocutaneous syndromes)
• genetic disorders associated with multiple tumours – for example, tuberous sclerosis and neurofibromatosis
• conditions that affect stomach acid, such as pernicious anaemia and chronic atrophic gastritis
• age – although NETs can occur at any age, there are some age groups that specific NETs can occur in, such as appendiceal NETs in a younger age group.

Some people have hereditary conditions that may predispose them to forming a NET. These people should be surveilled for syndrome-specific diagnosis and monitored annually. Hereditary conditions include (Rogel Cancer Centre University of Michigan 2021):

• multiple endocrine neoplasia (MEN)
  • MEN-1 – at risk of developing pituitary adenomas (PitNETs), pancreatic neuroendocrine tumours (pNETs) and hyperparathyroidism
  • MEN type 2A (RET gene mutation) – at risk of developing medullary thyroid cancer, hyperparathyroidism and phaeochromocytoma, noting that some families will only express medullary thyroid cancer
  • MEN type 2B (also known as MEN-3) (RET) – at risk of developing medullary thyroid cancer at a very young age, pheochromocytoma and mucosal neuromas of the lips, tongue and eyelids
  • MEN type 4 (CDKN1B gene mutation) – at risk of developing pituitary tumours, pancreatic NETs and hyperparathyroidism
  • MEN type 5 (MAX) – at risk of developing phaeochromocytomas and pituitary NETs
• VHL– at risk of developing pheochromocytomas, central nervous system and retinal haemangioblastomas, inner ear tumours, kidney tumours and pancreatic NETs
• succinate dehydrogenase gene complex (SDHA/B/C/D) mutations / paraganglioma syndrome – increased risk of phaeochromocytomas, paragangliomas, stomach gastrointestinal stromal tumours, kidney cancers and PitNETs
• fumarate hydratase mutations, which cause hereditary leiomyomatosis and renal cell carcinoma syndrome characterised by renal carcinomas, benign smooth muscle tumours of the skin and uterus and, occasionally, phaeochromocytoma and paragangliomas
• TMEM127 mutations – increased risk of phaeochromocytomas, paragangliomas and renal cell cancers.

For more information visit:

• phaeochromocytoma/paraganglioma panel testing
• VHL disease risk management

Increased awareness among health professionals is paramount to enable early diagnosis. There is often a prolonged delay in diagnosis because features are non-specific. General practitioners should have a strong clinical suspicion of patients who present with a combination of symptoms, or persistent symptoms (see section 2.1 for symptoms).

The following signs and symptoms for NETs are not specific, but where persistent or suspicious further investigation could be warranted:

• abdominal pain
• bloating
• repeated dry flushing on the face and neck
• diarrhoea, even while not eating
• wheezing/bronchoconstriction (asthma-like symptoms)
• episodes of hypotension or palpitations
• unexplained right-sided heart disease
• unexplained weight loss
• fatigue.

For gastrointestinal NETs, more specific symptoms for the NET location are:

• watery diarrhoea
• cramping
• intermittent abdominal pain
• flushing
• asthma-like wheezing
• bowel obstruction
• flushing, diarrhoea and dyspnoea – classic triad symptoms are seen in less than 20 per cent of cases.

For pancreatic NETs, more specific symptoms for the NET location are:

• epigastric or back pain
• peptic ulcer disease
• diarrhoea
• intermittent hypoglycemic episodes (low blood sugar)
• diabetes
• rash.

For lung NETs, more specific symptoms for the NET location are:

• wheezing
• cough
• dyspnoea
• haemoptysis
• recurrent chest infections/pneumonia.

For paragangliomas and phaeochromocytomas, more specific symptoms for the NET location are:

• hypertension, often paroxysmal
• headache
• heavy sweating for no known reason
• a strong, fast or irregular heartbeat
• tremor
• pallor.

The presence of multiple signs and symptoms indicates an increased risk of NETs.

General practitioner examinations and investigations should include:

• taking of a medical history and a physical examination
• full blood count, B12 and serum iron, liver function tests (LFTs) and renal function, thyroid function, calcium, cholesterol and C-reactive protein (CRP)
• imaging tests, such as ultrasound, chest x-ray (CXR), computed tomography (CT) scans
• referral for endoscopy/colonoscopy or bronchoscopy depending on imaging result.
• Tumour markers – for example, chromogranin A and 24-hour urinary 5HIAA – should be used with caution in the pre-diagnosis stage given the number of common false positives (e.g. chromogranin A may be falsely elevated in the context of proton pump inhibitor usage or renal failure).

See table 1 for specific investigations for the NET location.

If the cancer diagnosis is confirmed or the results are inconsistent or indeterminate, the general practitioner must refer the patient to an appropriate specialist (e.g. medical oncologist, gastroenterologist or respiratory physician) to make the diagnosis.

Patients should be enabled to make informed decisions about their choice of specialist and health service. General practitioners should make referrals in consultation with the patient after considering the clinical care needed, cost implications (see referral options and informed financial consent on page 9), waiting periods, location and facilities, including discussing the patient’s preference for health care through the public or the private system.

Referral for suspected or diagnosed NETs should include the following essential information to accurately triage and categorise the level of clinical urgency:

• important psychosocial history and relevant medical history
• family history, current symptoms, medications and allergies
• results of current clinical investigations (imaging and pathology reports)
• results of all prior relevant investigations
• notification if an interpreter service is required.

Many services will reject incomplete referrals, so it is important that referrals comply with all relevant health service criteria.

If access is via online referral, a lack of a hard copy should not delay referral.

The specialist should provide timely communication to the general practitioner about the consultation and should notify the general practitioner if the patient does not attend appointments.

Aboriginal and Torres Strait Islander patients will need a culturally appropriate referral. To view the optimal care pathway for Aboriginal and Torres Strait Islander people with cancer and the corresponding quick reference guide, visit the Cancer Australia website.

Download the consumer resources – Checking for cancer and Cancer from the Cancer Australia website.

The treatment team, after taking a thorough medical history and making a thorough medical examination of the patient, should undertake the following investigations under the guidance of a specialist.

• Biochemical markers – measurement of serum chromogranin A may be appropriate. Specific hormonal assessment will be depend on symptomology of the primary NET.
• Anatomical (e.g. CT, MRI) and functional imaging (68Ga-DOTATATE PET/CT, 18F-FDG PET) as indicated.
• Biopsy – histopathological diagnosis (grade and differentiation). Biopsies should be reviewed by a pathologist with experience in NETs.

Grading and staging is a critical element in treatment planning and should be clearly documented in the patient’s medical record.

The pathological biopsy should be reported or reviewed by a pathologist with expertise in NETs. NETs are graded pathologically based on the mitotic count (expressed as mitoses per 2mm2), the Ki-67 proliferative index and the presence of necrosis. Different cut-offs of Ki-67 index and mitotic rate are used to grade tumours of different sites, and for some sites the presence of necrosis increases the grade.

Other critical elements in the pathological reporting of NETs are:

• tumour site and size
• multifocality
• lymphovascular and perineural invasion
• extent of local invasion
• surgical margins
• nodal status
• presence of background disease.

In addition to mitotic count, Ki-67 index and the reporting of tumour necrosis (subject to site), which are considered mandatory in all NETs, other ancillary tests that may be useful in selected circumstances include (Perren et al. 2017):

• DAXX/ATRX immunohistochemistry (loss favours pancreatic origin)
• p53/pRb immunohistochemistry (p53 mutation and loss of RB1 favours NEC over NET)
• SSTR2 (may be used to predict positivity on 68Ga-DOTATATE PET/CT scans)
• MGMT immunohistochemistry or promoter methylation (potential predictor of response to temozolomide)
• immunohistochemistry for pancreatic hormone expression in context of a clinical hormonal syndrome (e.g. insulin and glucagon)
• SDHB immunohistochemistry (loss of expression makes syndromic disease due to germline mutation of one of the SDH genes highly likely).

See figure 4 (below) for classification of neuroendocrine tumour with corresponding imaging features and treatment options.

* GaTate: 68Ga-DOTATATE PET/CT; a specialised Gallium 68-dotatate tracer is injected and binds to somatostatin receptors on the cell surface of neuroendocrine cells (I-Med Radiology Network 2020). The presence of these receptors enables appropriate treatment choice.

* FDG: 18F-FDG PET/CT: The most common radiotracer is F-18 fluorodeoxyglucose (FDG), a molecule similar to glucose. Cancer cells are more metabolically active and may absorb glucose at a higher rate. 18-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) avidity in NETs has been associated with higher grade disease (Chan et al. 2020).

The presence of active uptake of 18F-FDG PET/CT enables appropriate treatment choice.
Source: Hofman & Hicks 2012

Classifying NETs into one of the 4 stages is site-specific and should be in the form of the tumour, nodes and metastases (TNM) system – refer to the 8th edition of the American Joint Committee on Cancer’s Staging Manual.

Staging for NETs may involve these tests:

• CT scan
• MRI
• 68Ga-DOTATATE PET/CT
• 18F-FDG PET/CT
• 18F-DOPA (for metastatic MTC, and for certain phaeochromocytomas)
• I-123-MIBG or I-131 MIBG imaging (for phaeochromocytomas and paragangliomas) – choice of imaging depends on availability and local preference.

Note: Phaeochromocytomas/paragangliomas should never be biopsied

More information

Visit the Cancer Institute New South Wales website for information about understanding the stages of cancer.

Patient performance status is a central factor in cancer care and should be clearly documented in the patient’s medical record.

Performance status should be measured and recorded using an established scale such as the Karnofsky scale or the Eastern Cooperative Oncology Group (ECOG) scale. The ECOG scale is the most used scale for NETs.

Patients should be encouraged to participate in research or clinical trials where available and appropriate.

For more information visit the Cancer Australia website.

The intent of treatment can be defined as one or more of the following:

• curative
• anti-cancer therapy to improve quality of life and/or longevity without expectation of cure
• symptom palliation.

The treatment intent should be established in a multidisciplinary setting, documented in the patient’s medical record and conveyed to the patient and carer as appropriate.

The potential benefits need to be balanced against the morbidity and risks of treatment.

The lead clinician should discuss the advantages and disadvantages of each treatment and associated potential side effects with the patient and their carer or family before treatment consent is obtained and begins so the patient can make an informed decision. Supportive care services should also be considered during this decision-making process. Patients should be asked about their use of (current or intended) complementary therapies (see Appendix D).

Timeframes for starting treatment should be informed by evidence-based guidelines where they exist. The treatment team should recognise that shorter timeframes for appropriate consultations and treatment can promote a better experience for patients.

Initiate advance care planning discussions with patients before treatment begins (this could include appointing a substitute decision-maker and completing an advance care directive). Formally involving a palliative care team/service may benefit any patient, so it is important to know and respect each person’s preference (AGDH 2021).

Early referral to palliative care can improve the quality of life for people with cancer and in some cases may be associated with survival benefits (Haines 2011; Temel at al. 2010; Zimmermann et al. 2014). This is particularly true for cancers with poor prognosis.

The lead clinician should ensure patients receive timely and appropriate referral to palliative care services. Referral should be based on need rather than prognosis. Emphasise the value of palliative care in improving symptom management and quality of life to patients and their carers.

The ‘Dying to Talk’ resource may help health professionals when initiating discussions with patients about future care needs (see ‘More information’).

Ensure that carers and families receive information, support and guidance about their role in palliative care (Palliative Care Australia 2018).

Patients, with support from their family or carer and treating team, should be encouraged to consider appointing a substitute decision-maker and to complete an advance care directive.

Refer to step 6 for a more detailed description of managing patients with recurrent, residual or metastatic disease.

More information

These online resources are useful:

• Advance Care Planning Australia
• Care Search
• Dying to Talk
• the Palliative Care resource kit
• Palliative Care Australia (for patients and carers).

The team should support the patient to participate in research or clinical trials where available and appropriate. Many emerging treatments are only available on clinical trials that may require referral to certain trial centres.

For more information visit the Cancer Australia website.

The transition from active treatment to post-treatment care is critical to long-term health. In some cases, people will need ongoing, hospital-based care, and in other cases a shared follow-up care arrangement with their general practitioner may be appropriate. This will vary depending on the type and stage of cancer and needs to be planned.

Shared follow-up care involves the joint participation of specialists and general practitioners in the planned delivery of follow-up and survivorship care.

A shared care plan is developed that outlines the responsibilities of members of the care team, the follow-up schedule, triggers for review, plans for rapid access into each setting and agreement regarding format, frequency and triggers for communication.

Different scenarios need to be defined in transitioning from treatment. This will include resected patients, as well as patients with stable disease and unstable disease. Treatment is often ongoing and sequential.

A designated member of the multidisciplinary team (most commonly nursing or medical staff involved in the patient’s care) should provide the patient with a needs assessment and treatment summary and develop a survivorship care plan in conjunction with the patient. This should include a comprehensive list of issues identified by all members of the multidisciplinary team involved in the patient’s care and by the patient. These documents are key resources for the patient and their healthcare providers and can be used to improve communication and care coordination.

The treatment summary should cover, but is not limited to:

• the diagnostic tests performed and results
• diagnosis including stage, prognostic or severity score
• tumour characteristics
• treatment received (types and dates)
• current toxicities (severity, management and expected outcomes)
• interventions and treatment plans from other health providers
• potential long-term and late effects of treatment
• supportive care services provided
• follow-up schedule
• contact information for key healthcare providers.

Patients with NETs may need multiple courses of therapy over their lifetime, given the relapsing-remitting nature of NETs. Monitoring for late effects and second malignancies is required, and primary prevention and risk minimisation strategies are encouraged.

Specific monitoring for sequelae of therapy should include:

• after surgery resulting in short gut syndrome – dietary intervention is required due to malabsorption
• after and/or during continuous systemic treatment – review and physical examination every three to six months for two years, and every six to 12 months subsequently (ESMO 2014; Knigge et al. 2017)
• follow-up echocardiography annually for patients who have carcinoid heart disease (they have an increased risk of cardiac valve dysfunction)
• after PRRT monitoring of renal function and risk of myelodysplastic syndrome
• monitoring for recurrence after surgery (Singh et al. 2018) – there are no specific follow-up guidelines because resected GEP-NETs are complex and emphasise closer follow-up for the first three years; however, it is recommended for follow-up to be for up to 10 years for fully resected small-bowel and pNETs and also identify clinical situations where follow-up is not required.

Responsibility for follow-up care should be agreed between the lead clinician, the general practitioner, relevant members of the multidisciplinary team and the patient. This is based on guideline recommendations for post-treatment care, as well as the patient’s current and anticipated physical and emotional needs and preferences.

Evidence comparing shared follow-up care and specialised care indicates equivalence in outcomes including recurrence rate, cancer survival and quality of life (Cancer Research in Primary Care 2016).

Ongoing communication between healthcare providers involved in care and a clear understanding of roles and responsibilities is key to effective survivorship care.

In particular circumstances, other models of post-treatment care can be safely and effectively provided such as nurse-led models of care (Monterosso et al. 2019). Other models of post-treatment care can be provided in these locations or by these health professionals:

• in a shared care setting
• in a general practice setting
• by non-medical staff
• by allied health or nurses
• in a non-face-to-face setting (e.g. by telehealth).

A designated member of the team should document the agreed survivorship care plan. The survivorship care plan should support wellness and have a strong emphasis on healthy lifestyle changes such as a balanced diet, a non-sedentary lifestyle, weight management and a mix of aerobic and resistance exercise (COSA 2018; Hayes et al. 2019).

This survivorship care plan should also cover, but is not limited to:

• what medical follow-up is required (surveillance for recurrence or secondary and metachronous cancers, screening and assessment for medical and psychosocial effects)
• model of post-treatment care, the health professional providing care and where it will be delivered
• care plans from other health providers to manage the consequences of cancer and cancer treatment
• wellbeing, primary and secondary prevention health recommendations that align with chronic disease management principles
• rehabilitation recommendations
• available support services
• a process for rapid re-entry to specialist medical services for suspected recurrence.

Prognosis

Many patients have a promising prognosis from low-grade NETs. Patients may need follow-up over a long period to monitor for progression or recurrence. Patients with incurable low-grade (grade 1) disease often survive for many (10+) years (NECA 2019a).

In contrast, patients with high-grade (grade 3) disease (high tumour burden that is aggressive) have poor progression-free survival, with an average survival time measured in the range of many months to a few years, despite best treatment (NECA 2019a).

There is a big variation in prognosis – there is a lot of variation in outcomes and no ‘magic number’ for a particular patient. Some patients find discussion of ranges in prognosis (best case / worse case / expected scenarios) very helpful, while others do not (NECA 2019a).

Prognoses based on the available information are a rough estimate because new treatments and insights can improve care and hence prognosis for all NET patients (NECA 2019a).

Table 2 provides a general guide for evaluating prognostic factors and outcomes for patients with NETs.

Table 2: Prognostic factors and outcomes for patients with neuroendocrine tumours

Survivors generally need regular follow-up, often for at least 10 years for most resected NETs and lifelong for those with incurable disease. The survivorship care plan therefore may need to be updated to reflect changes in the patient’s clinical and psychosocial status and needs.

Processes for rapid re-entry to hospital care should be documented and communicated to the patient and relevant stakeholders.

Care in the post-treatment phase is driven by predicted risks (e.g. the risk of recurrence, developing late effects of treatment and psychological issues) as well as individual clinical and supportive care needs. It is important that post-treatment care is based on evidence and is consistent with guidelines. Not all people will require ongoing tests or clinical review and may be discharged to general practice follow-up.

The lead clinician should discuss (and general practitioner reinforce) options for follow-up at the start and end of treatment. It is critical for optimal aftercare that the designated member of the treatment team educates the patient about the symptoms of recurrence.

General practitioners (including nurses) can:

• connect patients to local community services and programs
• manage long-term and late effects
• manage comorbidities
• provide wellbeing information and advice to promote self-management
• screen for cancer and non-cancerous conditions.

More information

Templates and other resources to help with developing treatment summaries and survivorship care plans are available from these organisations:

• Australian Cancer Survivorship Centre
• Cancer Australia – Principles of Cancer Survivorship
• Cancer Council Australia and states and territories
• Clinical Oncology Society of Australia – Model of Survivorship Care
• eviQ – Cancer survivorship: introductory course
• mycareplan.org.au
• South Australian Cancer Service – Statewide Survivorship Framework resources
• American Society of Clinical Oncology – guidelines.

Comparative data from randomised controlled trials are lacking in NETs. Therefore, treatment decisions are based on phase II trial data and patient/physician preferences. However, increasing active research and prospective trials are becoming available.

Support cancer survivors to participate in research or clinical trials where they are available and appropriate. These might include studies to understand survivors’ issues, to better manage treatment side effects, or to improve models of care and quality of life.

For more information visit the Cancer Australia website.

Some patients will have metastatic disease on initial presentation. Others may present with symptoms of recurrent disease after a previous cancer diagnosis. Access to the best available therapies, including clinical trials, as well as treatment overseen by a multidisciplinary team, are crucial to achieving the best outcomes for anyone with metastatic disease.

Signs and symptoms will depend on the type of cancer initially diagnosed and the location of metastatic disease. They may be discovered by the patient or by surveillance in the post-treatment period. New symptoms or changes in symptoms may include (Cancer.Net 2019):

• increase in facial flushing
• increased diarrhoea
• wheezing
• heart problems such as palpitations, difficulty breathing, becoming easily tired
• increased abdominal pain
• bloating
• bone pain.

In a suspected recurrence or progression, re-staging of relapsed or refractory disease should be performed with:

• bloods
• 24-hour urinary 5HIAA if carcinoid syndrome
• contrast CT of chest/abdomen/pelvis or MRI
• Ga-68-DOTATATE PET/CT (or similar)
• FDG PET/CT.

Managing metastatic disease is complex and should therefore involve all the appropriate specialties in a multidisciplinary team including palliative care where appropriate. From the time of diagnosis, the team should offer patients appropriate psychosocial care, supportive care, advance care planning and symptom-related interventions as part of their routine care. The approach should be personalised to meet the patient’s individual needs, values and preferences. The full complement of supportive care measures as described throughout the optimal care pathway and in Appendices A, B and C should be offered to assist patients and their families and carers to cope. These measures should be updated as the patient’s circumstances change.

Survivorship care should be considered and offered at an early stage. Many people live with advanced cancer for many months or years. As survival is improving in many patients, survivorship issues should be considered as part of routine care. Health professionals should therefore be ready to change and adapt treatment strategies according to disease status, prior treatment tolerance and toxicities and the patient’s quality of life, in addition to the patient’s priorities and life plans.

If there is an indication that a patient’s cancer has returned or progressed, care should be provided under the guidance of a treating specialist. Each patient should be referred back to the original multidisciplinary team. The multidisciplinary team may include new members such as palliative care specialists.

Treatment will depend on the location, extent of recurrent or residual disease, previous management and the patient’s preferences.
In managing people with NETs, treatment may include these options (refer to Step 4 for further detail):

• change in SSA – regimen and/or dose
• PRRT
• localised progression – consider liver-directed therapy and radiation therapy for bone metastasis
• widespread and systemic recurrence – consider systemic therapy – chemotherapy, targeted therapies, immunotherapy (remains investigational)
• pre-screen patient for any currently recruiting or imminent clinical trial
• palliation.

The potential goals of treatment should be discussed, respecting the patient’s cultural values. Wherever possible, written information should be provided.

Encourage early referral to clinical trials or accepting an invitation to participate in research.

Carcinoid syndrome

When GEP-NETs metastasise, the most common site for metastatic tumours (‘secondaries’) is the liver. Other areas of spread can include the bones, the lungs and the lymphatic system. Many metastatic NETs can cause symptoms due to over-production of hormones. The most common is carcinoid syndrome, which can occur in approximately 10 per cent of patients and is caused when an excess of hormones such as serotonin, histamine and somatostatin are produced (NORD 2021). The symptoms of carcinoid syndrome vary and can often be highly individual.

Typical symptoms include (NECA 2020a):

• flushing
• diarrhoea
• faecal urgency
• wheezing
• abdominal pain
• pellagra (niacin deficiency). Although rare, pellagra presents as a rash, dark pigmentation on the skin, a swollen mouth and a bright red tongue.

Patients can experience vomiting and diarrhoea, headache, fatigue, depression, disorientation, confusion or memory loss.

Carcinoid crisis

Sometimes patients with functioning NETs may suffer a particularly severe episode of carcinoid syndrome triggered by stressors, general anaesthetic or treatments. Early recognition of a carcinoid crisis enables preventative measures. Symptoms include (NECA 2020a):

• intense flushing
• diarrhoea
• abdominal pain
• wheezing
• palpitations
• low or high blood pressure
• altered mental state and, in extreme cases, coma.

Without treatment, the complications can be life-threatening, but if the patient is having any procedures the NET specialist should ensure the patient is comprehensively monitored and may give an infusion of an SSA (octreotide) as a preventative measure as per institutions policy.

NeuroEndocrine Cancer Australia provides a fact sheet named Carcinoid Crisis and Anaesthesia , and a wallet card for patients, which gives advice on treatment in the event of carcinoid crisis.

Carcinoid heart disease

Up to 20 per cent of patients with carcinoid syndrome present with carcinoid heart disease and, without treatment, can develop right heart failure (NECA 2020b). With SSAs, the progression of carcinoid heart disease is significantly slowed and other symptoms of heart failure may be managed with diuretics. Some patients with carcinoid heart disease may be suitable for cardiac surgery to replace the leaking valves (NECA 2020b). ECG and chest x-ray may provide clues to the diagnosis of carcinoid heart disease, but the most sensitive test is echocardiography of the heart. Echocardiography should be performed regularly to monitor the function of the heart in patients with functional neuroendocrine cancers (NECA 2020b). Urinary 5HIAA is a useful companion biomarker that measures serotonin excretion and also ProBNP (Grozinsky-Glasberg et al. 2015). While early valvular replacement can be complex, this should be managed in a specialist centre with experience in managing carcinoid valvular disease.

Advance care planning is important for all patients with a cancer diagnosis but especially those with advanced disease. Patients should be encouraged to think and talk about their healthcare values and preferences with family or carers, appoint a substitute decision-maker and consider developing an advance care directive to convey their preferences for future health care in the event they become unable to communicate their wishes (AGDH 2021).

More information

Refer to section 4.3 ‘More information’ for links to resources.

Refer patients and carers to Advance Care Planning Australia or to the Advance Care Planning National Phone Advisory Service on 1300 208 582.

Early referral to palliative care can improve the quality of life for people with cancer and in some cases may be associated with survival benefits (Haines 2011; Temel et al. 2010; Zimmermann et al. 2014). The treatment team should emphasise the value of palliative care in improving symptom management and quality of life to patients and their carers. Refer to section 4.3 for more detailed information.

The lead clinician should ensure timely and appropriate referral to palliative care services. Referral to palliative care services should be based on the patient’s need and potential for benefit, not prognosis.

More information

Refer to the end of section 4.3 ‘Palliative care’ for links to resources.

The treatment team should support the patient to participate in research and clinical trials where available and appropriate.

For more information visit the Cancer Australia website.

If the treatment team does not include a palliative care member, the lead clinician should consider referring the patient to palliative care services, with the general practitioner’s engagement. This may include inpatient palliative unit access (as required).

The multidisciplinary team may consider seeking additional expertise from these professionals:

• clinical psychologist
• clinical nurse specialist or practitioner
• social worker
• palliative medicine specialist
• pain specialist
• pastoral or spiritual carer
• bereavement counsellor
• music therapist
• art therapist
• cultural expert
• Canteen for children of parents with cancer.

The team might also recommend that patients access these services:

• home and community-based care
• specialist community palliative care workers
• community nursing.

If the patient does not already have an advance care directive in place, a designated member of the treatment team should encourage them to develop one in collaboration with their family or carer (AGDH 2021).

It is essential for the treatment team to consider the appropriate place of care, the patient’s preferred place of death and the support needed for the patient, their family and carers.

The treatment team should also ensure that carers and families receive the information, support and guidance about their role according to their needs and wishes (Palliative Care Australia 2018).

More information

The treatment team can refer patients and carers to these resources:

• Palliative Care Australia
• Advance Care Planning Australia 

Clinical trials may help improve palliative care and in managing a patient’s symptoms of advanced cancer (Cancer Council Victoria 2019). The treatment team should support the patient to participate in research and clinical trials where available and appropriate.

For more information visit the Cancer Australia website.

See ‘Resource list’ for additional clinical trial databases.

Cancer is the third leading cause of burden of disease for Aboriginal and Torres Strait Islander people. While Australia’s cancer survival rates are among the best in the world, Aboriginal and Torres Strait Islander people continue to experience a different pattern of cancer incidence and significant disparities in cancer outcomes compared with non-Indigenous Australians.

For Aboriginal and Torres Strait Islander people, health and connection to land, culture, community and identity are intrinsically linked. Health encompasses a whole-of-life view and includes a cyclical concept of life–death–life.

The distinct epidemiology of cancer among Aboriginal and Torres Strait Islander people, and unique connection to culture, highlight the need for a specific optimal care pathway for Aboriginal and Torres Strait Islander people with cancer. Ensuring this pathway is culturally safe and supportive is vital to tackling the disparities for Aboriginal and Torres Strait Islander people.

Published in 2018, the Optimal care pathway for Aboriginal and Torres Strait Islander people with cancer provides guidance to health practitioners and service planners on optimal care for Aboriginal and Torres Strait Islander people with cancer across the cancer continuum.

In addition to the key principles underpinning tumour-specific pathways, these are the key concepts that are fundamental to Aboriginal and Torres Strait Islander health:

• providing a holistic approach to health and wellbeing
• providing a culturally appropriate and culturally safe service
• acknowledging the diversity of Aboriginal and Torres Strait Islander peoples
• understanding the social determinants and cultural determinants of health (Cancer Australia 2015).

To view the Optimal care pathway for Aboriginal and Torres Strait Islander people with cancer, visit the Cancer Australia website . To view the consumer resources – Checking for cancer and Cancer, visit the Cancer Australia website 

For people from culturally diverse backgrounds in Australia, a cancer diagnosis can come with additional complexities, particularly when English proficiency is poor. In many languages there is not a direct translation of the word ‘cancer’, which can make communicating vital information difficult.

Perceptions of cancer and related issues can differ greatly in people from culturally diverse backgrounds and this can affect their understanding and decision making after a cancer diagnosis. In addition to different cultural beliefs, when English language is limited there is potential for miscommunication of important information and advice, which can lead to increased stress and anxiety for patients.

A professionally trained interpreter (not a family member or friend) should be made available when communicating with people with limited English proficiency. Navigation of the Australian healthcare system can pose problems for those with a non-Anglo culture, and members of the treatment teams should pay particular attention to supporting these patients.

The Australian Cancer Survivorship Centre has developed a glossary of more than 700 cancer terms in nine different languages. The multilingual glossary has been designed as a resource for professional translators, interpreters and bilingual health professionals working in the cancer field. The glossary is a unique tool that enables language professionals with access to accurate, consistent and culturally appropriate terminology.

Visit the Peter Mac website to see the glossary.

Disability, which can be physical, intellectual or psychological, may have existed before the cancer diagnosis or may be new in onset (occurring due to the cancer treatment or incidentally). Adjusting to life with a disability adds another challenge to cancer care and survivorship.

Several barriers prevent people with disabilities from accessing timely and effective health care (AIHW 2017):

• physical limitations
• competing health needs
• the trauma of undergoing invasive procedures
• potential barriers associated with obtaining informed consent
• failure to provide assistance with communication
• lack of information
• discriminatory attitudes among healthcare

In caring for people with disabilities and a cancer diagnosis, the Australian Institute of Health and Welfare disability flag should be used at the point of admittance to correctly identify and meet the additional requirements of a person with disability. Facilities should actively consider access requirements, and health practitioners should make reasonable adjustments where required.

Patients aged between seven and 65 years who have a permanent or significant disability may be eligible for support or funding through the National Disability Insurance Scheme (National Disability Insurance Agency 2018). More information can be found on the NDIS website. Patients aged 65 years or older (50 years or older for Aboriginal or Torres Strait Islander people) may be eligible for subsidised support and services through aged care services. An application to determine eligibility can be completed online over the phone. More information can be found at the My Aged Care website .

More information

‘Talking End of Life’ is a resource that shows how to teach people with intellectual disability about end of life. It is designed for disability support workers but is also helpful for others including families, health professionals and educators.

To view the resource, visit the Talking End of Life website 

Planning and delivering appropriate cancer care for older people can present a number of challenges. This could also be true for frail people or those experiencing comorbidities. Effective communication between oncology and geriatrics departments will help facilitate best practice care, which takes into account physiological age, complex comorbidities, risk of adverse events and drug interactions, as well as the implications of cognitive impairment on suitability of treatment and consent (Steer et al. 2009).

At a national interdisciplinary workshop convened by the Clinical Oncology Society of Australia, it was recommended that people over the age of 70 undergo some form of geriatric assessment, in line with international guidelines (COSA 2013; palliAGED 2018). Screening tools can be used to identify those patients in need of a comprehensive geriatric assessment (Decoster et al. 2015). This assessment can be used to help determine life expectancy and treatment tolerance and guide appropriate referral for multidisciplinary intervention that may improve outcomes (Wildiers et al. 2014).

Frailty is not captured through traditional measures of performance status (e.g. ECOG) and includes assessment in the domains of:

• function
• comorbidity
• presence of geriatric syndromes
• nutrition
• polypharmacy
• cognition
• emotional status
• social

In recent years, adolescent and young adult oncology has emerged as a distinct field due to lack of progress in survival and quality-of-life outcomes.

The significant developmental change that occurs during this life stage complicates a diagnosis of cancer, often leading to unique physical, social and emotional effects for young people at the time of diagnosis and throughout the cancer journey (Smith et al. 2012).

In caring for young people with cancer, akin to the comorbidities that require specific care in the older cancer population, the treatment team needs to pay careful attention to promoting normal development (COSA 2014). This requires personalised assessments and management involving a multidisciplinary, disease-specific, developmentally targeted approach that adheres to the following principles:

• understanding the developmental stages of adolescence and supporting normal adolescent health and development alongside cancer management
• understanding and supporting the rights of young people
• communication skills and information delivery that are appropriate to the young person
• meeting the needs of all involved, including the young person, their carers and their family
• working with educational institutions and workplaces
• considering survivorship and palliative care

An oncology team caring for an adolescent or young adult with cancer should be able to demonstrate these specific areas of expertise:

• be able to ensure access to expert adolescent and young adult health providers who have knowledge specific to the biomedical and psychosocial needs of the population
• understand the biology and current management of the disease in the adolescent and young adult age group
• consider participating in research and clinical trials for each patient
• engage in proactive discussion and management of fertility preservation, late effects of treatment, ongoing need for contraception, and psychosocial and psychosexual needs
• provide treatment in an environment that is friendly to adolescents and young

In general, people from lower socioeconomic groups are at greater risk of poor health, have higher rates of illness, disability and death, and live shorter lives than those from higher socioeconomic groups (AIHW 2016). People experiencing socioeconomic disadvantage are less likely to participate in screening programs, more likely to be obese, less likely to exercise and much more likely to smoke, which are all risk factors for cancer. In 2010–2014 age-standardised cancer incidence rates were higher in the lowest socioeconomic areas compared with the highest socioeconomic areas for all cancers combined (Cancer Australia 2019b).

Socioeconomic status and low health literacy are closely correlated. Therefore, effective communication with patients and carers is particularly important given the prevalence of low health literacy in Australia (estimated at 60 per cent of Australian adults) (ACSQHC 2014).

Consideration should be taken for cancer patients experiencing socioeconomic disadvantage to reduce their risk of being underserved for health care.

A diagnosis of cancer may present additional challenges to people who have pre-existing chronic mental health or psychiatric concerns, resulting in exacerbation of their mental health symptoms. This may include heightened anxiety, worsening depression or thoughts of self-harm.

As poor adjustment and coping can affect treatment decisions, people who are known to have a mental health diagnosis need psychosocial assessment in the oncology setting to formulate a plan for ongoing support throughout treatment.

Psychosocial support can assist with challenges in communicating with health professionals, enhance understanding of the treatment journey, ensure capacity for consent to treatment options and improve compliance with treatment requests. A referral for psychosocial support from a health professional to the psycho-oncology team can ensure these patients are provided with targeted interventions or referrals to community-based services that may mitigate problems associated with the impacts of social isolation that frequently accompany chronic mental ill-health.

Many patients with chronic mental health problems may be well known to external service providers. Psycho-oncology health professionals can form meaningful partnerships with existing service providers to optimise patient care throughout treatment and beyond.

Drug use disorders fall within the area of mental health conditions. People who are opiate dependent may have specific and individual requirements regarding pain management and their own preference for type of opiate prescribed or used.

People who identify as sexually or gender diverse may have unique needs following a cancer diagnosis. Sexually or gender diverse identities include (but are not limited to) people who identify as lesbian, gay, bisexual or transgender. There is no universally agreed upon initialism to describe this community, with other terms such as queer/questioning (Q), intersex (I), asexual (A) and pansexual

(P) often included, as well as a plus symbol (+) indicating inclusivity of other identities not explicitly mentioned. For the purposes of this document, this community is referred to as LGBTQI+.

Sexual orientation and gender identity are relevant across the entire spectrum of cancer care, from prevention to survivorship and end-of-life care. LGBTQI+ people are less likely to participate in cancer screening, and some segments of the LGBTQI+ community exhibit elevated rates of specific cancer risk factors – for example, higher rates of smoking and alcohol use. Regarding treatment, there may be unique factors relevant to LGBTQI+ people that may affect decision making. Additionally, the LGBTQI+ population experiences higher rates of anxiety, depression and

stressful life circumstances, and may be at risk of inferior psychosocial outcomes following a cancer diagnosis. LGBTQI+ people are also more likely to be estranged from their families of origin, and for older people, less likely to have adult children who may provide support and care.

Barriers to care for LGBTQI+ people include past negative interactions with healthcare systems, experiences or fear of discrimination and harassment in healthcare settings, assumptions of cisgender/heterosexual identity, lack of recognition or exclusion of same-sex partners from care, and a lack of relevant supportive care and information resources.

To provide safe and appropriate care for LGBTQI+ people with cancer, healthcare providers should:

• display environmental cues to show an inclusive and safe setting for LGBTQI+ patients
• avoid assumptions about the sexual orientation or gender identity of patients and their partners
• facilitate positive disclosure of sexual orientation or gender identity
• include same-sex/gender partners and families of choice in care
• be aware of relevant supportive care and information resources
• provide non-judgemental, patient-centred

Supportive care in cancer refers to the following five domains:

• the physical domain, which includes a wide range of physical symptoms that may be acute, relatively short lived or ongoing, requiring continuing interventions or rehabilitation
• the psychological domain, which includes a range of issues related to the patient’s mental health wellbeing and personal relationships
• the social domain, which includes a range of social and practical issues that will affect the patient, carer and family such as the need for emotional support, maintaining social networks and financial concerns
• the information domain, which includes access to information about cancer and its treatment, recovery and survivorship support services and the health system overall
• the spiritual domain, which focuses on the patient’s changing sense of self and challenges to their underlying beliefs and existential concerns (Palliative Care Victoria 2019).

Fitch’s (2000) model of supportive care recognises the variety and level of intervention required at each critical point as well as the need to be specific to the individual patient (Figure A1). The model targets the type and level of intervention required to meet patients’ supportive care needs.

Figure A1: Fitch’s tiered approach to supportive care

Consider a referral to a psychologist, psychiatrist, pastoral/spiritual care practitioner, social worker, specialist nurse or a relevant community-based program if the patient has these issues:

• displaying emotional cues such as tearfulness, distress that requires specialist intervention, avoidance or withdrawal
• being preoccupied with or dwelling on thoughts about cancer and death
• displaying fears about the treatment process or the changed goals of their treatment
• displaying excessive fears about cancer progression or recurrence
• worrying about loss associated with their daily function, dependence on others and loss of dignity
• becoming isolated from family and friends and withdrawing from company and activities that they previously enjoyed
• feeling hopeless and helpless about the effect that cancer is having on their life and the disruption to their life plans
• struggling to communicate with family and loved ones about the implications of their cancer diagnosis and treatment
• experiencing changes in sexual intimacy, libido and function
• struggling with the diagnosis of relapsed, refractory or advanced disease
• having difficulties quitting smoking (refer to Quitline on 13 7848) or with other drug and alcohol use
• having difficulties transitioning to palliative

Additional considerations that may arise for the multidisciplinary team include:

• support for the carer – encourage referrals to psychosocial support from a social worker, psychologist or general practitioner
• referral to an exercise physiologist or physiotherapist as a therapeutic approach to prevent and manage psychological health
• referral to wellness-after-cancer programs to provide support, information and offer

Complementary therapies may be used together with conventional medical treatments to support and enhance quality of life and wellbeing. They do not aim to cure the patient’s cancer. Instead, they are used to help control symptoms such as pain and fatigue (Cancer Council Australia 2019).

The lead clinician or health professional involved in the patient’s care should discuss the patient’s use (or intended use) of complementary therapies not prescribed by the multidisciplinary team to assess safety and efficacy and to identify any potential toxicity or drug interactions.

The lead clinician should seek a comprehensive list of all complementary and alternative medicines being taken and explore the patient’s reason for using these therapies and the evidence base. A transparent and honest discussion that is free from judgement should be encouraged.

While some complementary therapies are supported by strong evidence, others are not. For such therapies, the lead clinician should discuss their potential benefits and use them alongside conventional therapies (NHMRC 2014).

If the patient expresses an interest in using complementary therapies, the lead clinician should consider referring patients to health providers within the multidisciplinary team who have expertise in the field of complementary and alternative therapies (e.g. a clinical pharmacist, dietitian or psychologist) to assist them to reach an informed decision. Costs of such approaches should be part of the discussion with the patient and considered in the context of evidence of benefit.

The lead clinician should assure patients who use complementary therapies that they can still access a multidisciplinary team review and encourage full disclosure about therapies being used.

More information

• See the Clinical Oncological Society of Australia’s position statement Use of complementary and alternative medicine by cancer patients 

Advance Care Planning Australia

Advance Care Planning Australia provides national advance care planning resources for individuals, families, health professional and service providers. Resources include a national advisory service, information resources, a legal forms hub and education modules.

• Telephone: 1300 208 582
• Website

Australian Cancer Survivorship Centre

The Australian Cancer Survivorship Centre has developed information resources and events to help people move from initial treatment to post treatment and beyond, including those receiving maintenance treatments. While they do not provide clinical advice, they connect with a range of providers to enable improved care.

• Telephone: (03) 8559 6220
• Website

Australian Commission on Safety and Quality in Health Care

The Australian Commission on Safety and Quality in Health Care has developed a resource for patients and carers explaining the coordination of care that patients should receive from their health service during cancer treatment. The resource is called What to expect when receiving medication for cancer care.

Beyond Blue

Beyond Blue provides information about depression, anxiety and related disorders, as well as about available treatment and support services.

• Telephone: 1300 22 4636
• Website

Cancer Australia

Cancer Australia provides information for consumers, carers and their families including printed resources and video content.

• Website

Cancer Council’s Cancer Information and Support Service

Cancer Council 13 11 20 is a confidential telephone support service available to anyone affected by cancer. This service acts as a gateway to evidence-based documented, practical and emotional support available through Cancer Council services and other community organisations. Calls will be answered by a nurse or other oncology professional who can provide information relevant to a patient’s or carer’s situation. Health professionals can also access this service.

• Telephone: 13 11 20 – Monday to Friday, 9.00am to 5.00pm (some states have extended hours)
• Website

Cancer Council’s Cancer Connect

Cancer Connect is a free and confidential telephone peer support service that connects someone who has cancer with a specially trained volunteer who has had a similar cancer experience.

A Connect volunteer can listen with understanding and share their experiences and ways of coping. They can provide practical information, emotional support and hope. Many people newly diagnosed with cancer find this one-to-one support very beneficial.

For more information on Cancer Connect call Cancer Council on 13 11 20.

Canteen

Canteen helps adolescents, young adults and parents to cope with cancer in their family. Canteen offers individual support services, peer support services and a youth cancer service, as well as books, resources and useful links.

• Telephone: 1800 835 932 to talk to a health professional about information and support for young people or 1800 226 833 for other enquiries
• Website

Clinical trial information

For a collection of clinical trials available in Australia see the following sources of information:

• Cancer Australia
• Australian New Zealand Clinical Trials Registry
• Australasian Leukaemia and Lymphoma Group 
• ClinTrial Refer 
• Clinical Trials for an international view.

CanEAT pathway

A guide to optimal cancer nutrition for people with cancer, carers and health professionals.

• Education website
• Patient website 

Guides to best cancer care

The short guides help patients, carers and families understand the optimal cancer care that should be provided at each step. They include optimal timeframes within which tests or procedures should be completed, prompt lists to support patients to understand what might happen at each step of their cancer journey and to consider what questions to ask, and provide information to help patients and carers communicate with health professionals.

The guides are located on an interactive web portal, with downloadable PDFs available in multiple languages.

• Website

Look Good, Feel Better

A free national community service program, run by the Cancer Patients Foundation, dedicated to teaching cancer patients how to manage the appearance-related side effects caused by treatment for any type of cancer.

• Telephone: 1800 650 960
• Website

Quitline

Quitline is a confidential, evidence-based telephone counselling service. Highly trained Quitline counsellors use behaviour change techniques and motivational interviewing over multiple calls to help people plan, make and sustain a quit attempt.

Quitline is a culturally inclusive service for all, and Aboriginal counsellors are also available. Health professionals can refer patients to Quitline online or via fax.

• Telephone: 13 7848
• Website or the relevant website in your state or territory.

Australian Cancer Survivorship Centre

The Australian Cancer Survivorship Centre provides expertise in survivorship care, information, support and education. Its purpose is to support and enable optimal survivorship care.

• Telephone: (03) 8559 6220
• Website 

Australian Commission on Safety and Quality in Health Care

The Australian Commission on Safety and Quality in Health Care has developed a guide for clinicians containing evidence-based strategies to support clinicians to understand and fulfil their responsibilities to cancer patients.

This guide is particularly relevant to Steps 3 to 6 of the optimal care pathway. The guide is titled NSQHS Standards user guide for medication management in cancer care for clinicians

Cancer Australia

Information for health providers including guidelines, cancer learnings, cancer guides, reports, resources, videos, posters and pamphlets.

• Website 

Cancer Council Australia

Information on prevention, research, treatment and support provided by Australia’s peak independent cancer authority.

• Website 

CanEAT pathway

A guide to optimal cancer nutrition for people with cancer, carers and health professionals.

• Education website
• Patient website 

eviQ

A clinical information resource providing health professionals with current evidence-based, peer-maintained, best practice cancer treatment protocols and information relevant to the Australian clinical environment.

• Website 

National Health and Medical Research Council

Information on clinical practice guidelines, cancer prevention and treatment.

• Website 

NeuroEndocrine Cancer Australia

NeuroEndocrine Cancer Australia (NECA) is a not-for-profit medical charity providing support to Australians living with neuroendocrine cancers. NECA is governed by five pillars: patient support, education, research, awareness and advocacy. These pillars have been created to give neuroendocrine cancer patients, their families and friends, a voice in the community and access to the care and treatment they deserve.

• Telephone: 1300 287 363
• Website

We acknowledge the Traditional Owners of Country throughout Australia and their continuing connection to the land, sea and community. We pay our respects to them and their cultures and to Elders past, present and emerging.

This work is available from the Cancer Council website.

Published in June 2022.

ISBN: 978-1-76096-748-2

Cancer Council Victoria and Commonwealth Government 2022, Optimal care pathway for people with neuroendocrine tumours, 1st edn, Cancer Council Victoria, Melbourne.

Enquiries about this publication can be sent to optimalcare.pathways@cancervic.org.au.

Dr David Chan (Chair), Medical Oncologist, Royal North Shore Hospital and The University of Sydney, NSW

A/Prof. David Wyld (Deputy Chair), Medical Oncologist, Royal Brisbane and Women’s Hospital, Qld

Ms Julia Brancato, Optimal Care Pathways Lead, Cancer Council Victoria

A/Prof. Gabrielle Cehic AM, Nuclear Medicine Physician, The Queen Elizabeth Hospital, SA

Prof. Ray Chan, Nurse, Flinders University, SA

A/Prof. Roderick Clifton-Bligh, Endocrinologist, Royal North Shore Hospital, NSW

Ms Meredith Cummins, Project Officer /Clinical Research Associate / NET Nurse, NeuroEndocrine Cancer Australia

Prof. Anthony Gill, Pathologist, Royal North Shore Hospital, NSW

Dr Erin Laing, Senior Dietitian, Peter MacCallum Cancer Centre, Victoria

Ms Simone Leyden, CEO, NeuroEndocrine Cancer Australia

Dr Benjamin Loveday, Surgeon, Peter MacCallum Cancer Centre, Vic

Dr Bill MacDonald, Nuclear Medicine Physician, Fiona Stanley Hospital, WA

Dr Katrina Marshall, General Practitioner, Barrier Reef Medical, Qld

Mrs Amanda Stork, Consumer, NeuroEndocrine Cancer Australia

Prof. Gerald Fogarty, Radiation Oncologist, Icon Cancer Centre Revesby, NSW

Prof. Michael Michael, Medical Oncologist, Peter MacCallum Cancer Centre

Advance Care Planning Australia

Allied Health Professions Australia

Australasian Association of Nuclear Medicine Specialists

Australasian Chapter of Palliative Medicine, Royal Australia College of Physicians

Australasian Gastro-Intestinal Trials Group

Australian and New Zealand Society of Neuroradiology

Australian and New Zealand Society of Palliative Care

Australian Cancer Survivorship Centre

Australian College of Nursing

Australian Medical Association

Australian Society of Medical Imaging and Radiation Therapy

Cancer Nurses Society of Australia

Clinical Oncology Society of Australia – Gastrointestinal Cancers Group &

Neuroendocrine Tumours Group

Commonwealth Neuroendocrine Tumour Research Collaborative (CommNETs)

Gastroenterological Society of Australia

Interventional Radiology Society of Australasia

Medical Oncology Group of Australia

NeuroEndocrine Cancer Australia

Oncology Social Workers Australia and New Zealand

Royal Australasian College of Physicians