Diagnosis and staging

• Biochemical markers – measurement of serum chromogranin A may be appropriate. Specific hormonal assessment will 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.

Genetic testing

Approximately 10–15% of all pancreatic neuroendocrine tumors (pNETs) are associated with MEN-1 and up to 80 per cent of patients with MEN-1 will develop pNETs – refer to the optimal care pathway for people with NETs for more information.

Find out more about phaeochromocytoma/ paraganglioma panel testing, MEN-2 and von Hippel-Lindau disease risk management on the eviQ website <www.eviq.org.au/cancer-genetics/adult>.

Treatment planning

All newly diagnosed 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. The level of discussion may vary, depending on the patient’s clinical and supportive care factors.

Research and clinical trials

Consider enrolment where available and appropriate. Search for a trial <www.australiancancertrials.gov.au>.

Communication

The lead clinician’s responsibilities include:

• discussing a timeframe for diagnosis and treatment options with the patient and/or carer
• explaining the role of the multidisciplinary team in treatment planning and ongoing care
• encouraging discussion about the diagnosis, prognosis, advance care planning and palliative care while clarifying the patient’s wishes, needs, beliefs and expectations, and their ability to comprehend the communication
• providing appropriate information and referral to support services as required
• communicating with the patient’s GP about the diagnosis, treatment plan and recommendations from multidisciplinary meetings.

Checklist

• Diagnosis confirmed
• Full histology obtained
• Performance status and comorbidities measured and recorded
• Patient discussed at an MDM and decisions provided to the patient and/or carer
• Clinical trial enrolment considered
• Supportive care needs assessment completed and recorded and referrals to allied health services actioned as required
• Patient referred to support services (e.g. Cancer Council and NeuroEndocrine Cancer Australia) as required
• Treatment costs discussed with the patient and/or carer

Timeframe

Diagnostic investigations should be completed within 2 weeks of the initial specialist appointment.