1. An Audit of Activity and Pathways The Management of Neuroendocrine Tumours (NET) in Merseyside and Cheshire Jon Hayes, Ged Corcoran and Chris Holcombe Sept 2008

2. 2 NET Service or DGH NET Service DGH SurgeryEndocrinologyGen Medicine NET Clinician NET Clinician NET Clinician NET Clinician Further Investigations Therapy Biochem / Haematology Radiology Imaging Radionuclide Imaging Surgery Interventional Radiology Chemotherapy (DXRT) Medical Treatment Radionuclide Therapy I 131 MIBG or Y 90 Dotatate Immediate Post-Treatment Follow-up Long Term Follow-up and Surveillance NET MDT

3. 3 The NET Service is provided by a number of local clinicians who have developed a special interest in this rare but complex condition. Incidence is around 2 per 100,000, but five year survival is around 70% so prevalence is high. Most patients require life-long follow-up and surveillance. Because the service has developed gradually over several years, only an informal clinical pathway has existed up until now. Consequently, important aspects of the service are not adequately funded. Some aspects of the clinical management are funded through PbR, but significant elements should be considered for collective and/or specialised commissioning. Neuroendocrine tumours have not been subject to NICE Improving Outcomes Guidance. Merseyside and Cheshire NET Service

4. 4 NETs are usually diagnosed after investigation for non-specific symptoms (eg nausea, vomiting and diarrhoea) by physicians and surgeons in hospitals across the cancer network. Most carcinoid tumours arise in the appendix or small bowel. 269 out of 396 patients on the local NET database have carcinoid tumours (68%). Insulinomas, gastrinomas, phaeochromocytomas, glucogonomas, VIPomas and somatostatinomas account for the remaining 32%. NETs are associated with genetic syndromes such as Multiple Endocrine Neoplasia (MEN1 and 2), but most are sporadic isolated tumours. At the time of onward referral to the NET Service, many patients may already have had a laparotomy or biopsy to confirm the diagnosis. Diagnosis and Onward Referral

5. 5 Site of primary: Referrals Small Bowel18% Appendix14% Pancreas12% Unknown10% Colon2% Thyroid2% Other42% Source: NET database, Jan 2006 – May 2008. Jan 2006 – end May 2008 (29 months):New patients = 162 (67 per year) Known deaths = 66 (27 per year)

6. 6 Site of metastases: Metastatic Disease Liver76% Lymph12% Mesentery7% Bone7% Ovary2% Other6% Source: NET database, Jan 2006 – May 2008. Total exceeds 100% as some patients have multiple sites recorded. 53% of patients have metastatic disease.

7. 7 Referrals are made from DGHs to several clinicians with a special interest in NETs at the Royal Liverpool University Hospital and University Hospital Aintree. Referrals are received from trusts within the Merseyside and Cheshire Cancer Network, North Wales and the Isle of Man. Less frequent referrals are received from Lancashire, South Cumbria, Manchester and the rest of Britain. Patients are seen by the specialist clinicians in their outpatient clinics and then their cases are discussed at the NET Multidisciplinary Team Meeting (MDT). The NET MDT has developed in a gradual manner over several years and has met fortnightly since 2007. The meeting is chaired by Professor Vinjamuri, Consultant in Nuclear Medicine. The MDT has not had the benefit of an Oncologist (medical/clinical) for two years. It is likely that the MDT may need to be held weekly in the near future. Assessment by NET Clinicians

8. 8 Attendance at MDT by NET Clinicians

9. 9 Once referred into the NET Service, patients may require further diagnostic tests to assess the nature and extent of their disease. Biochemestry / Haematology –Full blood and urine endocrine profile - FBC –Renal function - Hepatic Function Imaging –Chest X-Ray –Contrast enhanced CT chest and abdomen –MR Radionuclide Imaging –I 123 MIBG +/- Single Photon Emission Computed Tomography –In 111 Octreotide +/- SPECT Additional Investigations

10. 10 Radionuclide Imaging Source: Peer Review Document, Dept of Nuclear Medicine, RLBUHT, 30 June 2008

11. 11 Hormone Therapy –Somatostatin analogues (12% of patients) –Patients receive monthly injections in the community co-ordinated by NET CNSs Chemotherapy (2%) –Co-ordinated by the most appropriate oncology centre for the patient (CCO, Christie, Glan Clwyd, Preston) Interventional Radiology –Radio-Frequency Ablation (1%) –Chemo-Embolisation (<1%) Radionuclide Therapies –I 131 MIBG (5%) –Y 90 Dotatate (8%) Figures in parentheses show proportion of patients (referred between Jan 2006 and May 2008) receiving these treatments recorded on the NET database. Specialist Non-Surgical Therapies

12. 12 Surgery Source: NET database, Jan 2006 – May 2008. 52% of patients have surgery. Location: Right Hemicolectomy21% Appendicectomy20% Small Bowel Resection18% Whipples/Pancreatectomy14% Liver Resection6% Other surgery21% Total100% UHA21% RLUH14% Other29% Not recorded36% Total100%

13. 13 Radionuclide Therapies Source: Peer Review Document, Dept of Nuclear Medicine, RLBUHT, 30 June 2008

14. 14 Patients are seen by the responsible clinician in outpatient clinics at the Royal and Aintree. On-going surveillance and management of recurrence Recurrence is common in this group of patients and follow- up continues for a number of years. Some patients are returned to their original referring network for surveillance. More locally, patients receive at least annual checks by NET clinicians. Post-Treatment Follow-up

15. 15 1.Peer review measures stipulate minimum attendance at 50% of meetings for core MDT members. 2.Lack of CCO input to MDT needs to be addressed. 3.What investigations and treatments can be undertaken by referring hospital? 4.Trials recruitment: Is it as comprehensive as we would like? 5.What are the indications for specialist investigations and therapies? 6.Need to clarify which parts of the pathway are funded and how. Need to prepare a case for collective/specialised commissioners to consider regarding the unfunded elements. 7.What is the scope for developing the service beyond its current boundaries? 8.What is the role of PET-CT in NETs? For Discussion