Optimal timeframes & summary

Evidence-based guidelines, where they exist, should inform timeframes. Treatment teams need to recognise that shorter timeframes for appropriate consultations and treatment can promote a better experience for patients. Three steps in the pathway specify timeframes for care (Figure 3). They are designed to help patients understand the timeframes in which they can expect to be assessed and treated, and to help health services plan care delivery in accordance with expert-informed time parameters to meet the expectation of patients. These timeframes are based on expert advice from the Neuroendocrine Tumours Working Group.

Timeframes for care for neuroendocrine tumours.

Step in pathway Care point Timeframe
Presentation, initial investigations and referral Signs and symptoms Presenting symptoms should be promptly and clinically triaged by a general practitioner
Initial investigations initiated by GP Where this is a strong suspicion of neuroendocrine tumours, investigations should be conducted within 2 weeks of the initial GP appointment
Referral to specialist All patients with a suspected or proven neuroendocrine tumour should be referred to an appropriate specialist within 1 week of completing initial investigations
Diagnosis, staging and treatment planning Diagnosis and staging Diagnostic investigations should be completed within 2 weeks of the initial specialist appointment.
Multidisciplinary team meeting and treatment planning Patients should be presented at an appropriate neuroendocrine tumour multidisciplinary meeting, with all appropriate investigation results, within 4 weeks of diagnosis to develop the patient’s management plan
Treatment Surgery Timeframe for surgery will be based on investigation and staging of the neuroendocrine tumour and surgery intent.
Localised radiation therapy Treatment should start as soon as possible for symptomatic patients.
Systemic radiation therapy (peptide receptor radionuclide therapy [PRRT]) When PRRT is necessary (for patients who have sufficient expression of somatostatin receptors that have progressed on somatostatin analogues or where initial PRRT is considered appropriate), treatment should start as soon as possible.
Systemic therapy When active treatment is considered necessary, treatment should start within 4 weeks of the treatment decision.
Liver directed therapy When active treatment is considered necessary, treatment should start within 4 weeks of the treatment decision.

Seven steps of the optimal care pathway

Step 1: Prevention and early detection

Step 2: Presentation, initial investigations and referral step

Step 3: Diagnosis, staging and treatment planning

Step 4: Treatment

Step 5: Care after initial treatment and recovery

Step 6: Managing refractory, relapsed, residual or progressive disease

Step 7: End-of-life care

Neuroendocrine neoplasms (NENs) are a family of tumours defined by specific morphological features. They are separated into neuroendocrine tumours (NETs) and neuroendocrine carcinomas (NECs) which, by definition, are high-grade. The general term to refer to NENs was NETs until very recently and it is still known by this name to most non-NEN specialists; therefore, NETs will be used as the preferred term for this optimal care pathway.

The incidence of NETs has historically been thought to be very low and consequently they were categorised as a ‘rare cancer’ (NECA 2021). This is no longer the case, with more than 5,000 new patients estimated to be diagnosed this year in Australia (Cancer Australia 2021). In 2021 it was estimated that 3.4 per cent of all new cancer diagnoses will be neuroendocrine cancers, and their incidence now exceeds cancers such as bladder, liver, ovarian, brain and kidney cancers (AIHW 2021).

The increase in the incidence of NETs may partly be attributed to greater awareness of the disease and the development of improved imaging techniques. In 2017, there were 4,228 new cases of NETs diagnosed in Australia compared with more than 5,000 estimated in 2021 (AIHW 2021). Both the incidence of NETs and the survival rates are increasing (Dasari et al. 2017). Earlier diagnosis, improved diagnostic procedures and optimal treatments are the keys to improved survival rates and ongoing symptom management and improved quality of life.

More than 10,000 Australians diagnosed between 2012 and 2016 currently live with NETs, and more than 22,000 people are still living who had been diagnosed with NETs in the 35 years from 1982 to 2016 (AIHW 2021). NETs are the second most prevalent gastrointestinal malignancy after colorectal (bowel) cancer (NECA 2021).

According to the latest Australian Institute of Health and Welfare (AIHW) figures, in 2021, the average Australian is estimated to have a one in 56 chance of being diagnosed with a NET by the age of 85 (AIHW 2021). Despite the surprisingly high occurrence and high morbidity rates, NETs remain under-represented in local cancer research.

Survival rates vary significantly depending on the grade and location of the primary tumour. However, the overall survival for NETs is almost always better than for carcinomas arising at the same site. For example, pancreatic NETs have a 69 per cent five-year survival compared with 6.7 per cent for the more common pancreatic adenocarcinoma (AIHW 2021).

NETs can occur in people of all ages, and affect men and women equally. The most common sites for NETs are the stomach, lung and pancreas (Michael et al. 2021).

This optimal care pathway covers gastrointestinal NETs, lung NETs (known as carcinoid tumours; not small cell lung cancer), thymic NETs, thyroid NETs (known as medullary carcinoma), paragangliomas and pheochromocytomas. Both high-grade and low-grade NETs will be considered in this pathway.