Endocrine Oncology (OE)

Currently, the department of endocrine oncology manages around 1,000 patients with diagnoses in the condition and receives around 1,000 referrals annually for patients with NETs of the abdomen and adrenal tumours, of which 300 are new individual patients with tumours that fall within the given definition. Patients are referred from our own healthcare region, as well as from other regions in the country and internationally. About 200 of these come to Uppsala, while the remaining referral patients are managed at the local hospital with our support, for example via joint MDK. There are well-established procedures around investigations and patient flows, as well as treatment strategies that comply with the specific conditions of the NHV.

Among other things, the hospital provides targeted radionuclide therapy (PRRT treatment) to patients from Region Central Sweden and Region North, Jönköping, Kalmar and Gotland. In addition to this, the hospital is also currently managing Ireland's patients who need PRRT treatment. Patients from other countries without PRRTs, for example Ukraine, Lithuania, Bulgaria, Russia, also refer occasional patients to us. Uppsala University Hospital regularly receives requests for second opinions from other parts of Sweden and from colleagues and patients from the rest of Europe and the world.

Certifications and European networks

Uppsala University Hospital is the only hospital in Sweden to be a member of the European Reference Network (ERN) EURACAN, for rare tumours in adults, domain 4 for neuroendocrine tumours. Among other things, membership requires fulfilment of the criteria set by the ERN in terms of number of new patients per year, patient management, research and education activities, and information systems for patients and their families. In order to apply for EURACAN membership, you also need approval and a letter of support from Socialstyrelsen stating that you fulfil the criteria before you are permitted to submit your application, which we received in 2016.

Since the start of the programme in 2009, endocrine oncology is still the only practice in Sweden to be certified as an ENETS Centre of Excellence. To fulfil the certification requirements, at least 100 new patients with abdominal NETs must be treated annually, and there are requirements for which specialist competences must be contracted (endocrinologist, oncologist, pathologist, radiologist, nuclear medicine specialist, endocrine surgeon, pancreatic surgeon, thoracic surgeon, pulmonologist and gastroenterologist). Agreements must also be in place with accredited biochemistry and genetics laboratories, cardiologists, transplant surgeons, radiation oncologists, palliative care consultants, pain management units, dietitians, counsellors and patient organisations. It monitors processing times, the existence of weekly MDKs and the unit's training and research activities. Morbidity and mortality must be reported for all types of treatments including surgery, chemotherapy, radiotherapy and targeted therapy. A patient examination must be carried out at least every two years. They want to know the number of pathological and radiological examinations performed and compliance with European guidelines. There must be regular meetings to develop the organisation as well as an annual internal audit. Annual monitoring of fulfilment of the criteria for certification is required and for ENETS CoE status, on-site audits are carried out regularly to ensure fulfilment of the criteria. Uppsala remains certified under this programme.

In endocrine oncology, we have about 500 cases/year and 2400 doctor visits. We provide around 250 PRRT treatments in a year. There are >400 PET scans per year, most of which are PET scans to identify somatostatin receptor positive tumours (DOTATOC-) PET. Approximately 30 PET scans are performed annually using the specific tracers 18F-CETO and 11C-hydroxy ephedrine (HED) for the mapping of adrenal tumours. Because Uppsala is the only PET centre in Sweden that performs these, a number of examinations are also performed on direct referral from, for example, Stockholm, Örebro and Umeå.

The RCC registry for GEP-NET (INCA-based registry), which includes both benign and malignant NETs in the abdomen, gives us high coverage of the patients we are responsible for, but because many patients come from other regions, there is generally low coverage across the country. The coverage rate for advanced NETs in the small intestine and pancreas for the Central Sweden Region has been around 90% in recent years.

Endocrine surgery

In surgery, a similar arrangement exists for advanced NET and adrenal patients on a regional hospital basis. In addition to this, we also operate on a significant proportion of the advanced NET and adrenal patients from Region Västerbotten's catchment area. The Section for Endocrine Surgery in the Surgery Division currently performs all procedures within the licence area at Uppsala University Hospital. For procedures such as advanced liver and pancreatic surgery, it is performed jointly with the section for liver-pancreatic surgery and when cardiopulmonary bypass is required for advanced adrenal surgery, this is performed jointly with the thoracic surgeons.

Surgery Division annually performs (average of the last 5 years):

• Total number of pancreatic surgeries about 180, of which 40 surgeries are due to pancreatic NET (20 of which are laparoscopic and robotic assisted surgery for pancreatic NET)
• Total number of liver resections about 190, of which about 5 due to NET
• Approximately 25 operations due to advanced small bowel NET (of which laparoscopic surgery approximately 15)
• Total number of operations due to adrenal tumours 30
  • Pheochromocytoma 7
  • Adrenocortical carcinoma (ACC) 4

All Uppsala's surgeries are registered in the national quality registers, including complication rates. SQRTPA registers adrenal surgery and INCA registers pancreatic surgery. Adrenal surgeries are also recorded in European ENS@T. The coverage of these registers is close to 100 per cent for patients operated on in Uppsala within the licence area.

Quality registry for surgical treatment of thyroid, parathyroid and adrenal diseases (sqrtpa.se)  

European Network for the Study of Adreanal Tumours (wildapricot.org)  

Thoracic surgery (carcinoid heart disease)

New patients with small bowel NET and elevated serotonin levels are screened with cardiac echo and NT-proBNP at diagnosis. If carcinoid heart disease is found, the patient is assessed by a cardiologist who assesses the need for medication and the frequency of echocardiography checks. Endocrine Oncology (OE) monitors NT-proBNP and the clinical symptoms at tumour checks and in event of deterioration a new echocardiographic examination and cardiologist assessment is done liberally. Patients who deteriorate to the point where valve surgery is necessary are first assessed for tumours at the NET MDK to ensure that the tumour disease is substantially under control. If the patient is considered suitable for surgery from the point of view of the tumour, the patient is registered for a thoracic round in which doctors from endocrine oncology, clinical physiologist, cardiologist and thoracic surgeon participate. If the patient is accepted for surgery, the OE organises the fastest possible preoperative investigation (coronary examination and dental cleaning) at the home location or in Uppsala. If it is unclear whether the patient is operable, the patient is called to the OE and the responsible thoracic surgeon and thoracic anaesthesiologist within the MDK team assess the patient on site. If surgery is decided on, it must usually be done quickly because the patient's clinical status can deteriorate rapidly. Occasionally, patients are hospitalised as severe heart failure patients with no known NET. Echocardiography may reveal severe carcinoid heart disease with severe valve involvement. Referral then goes to the OE for investigation and treatment in consultation with a cardiologist.

Patients are then operated on and initially cared for post-operatively at the thoracic centre. Aftercare is then usually provided at the local hospital. All patients who undergo surgery are registered in the Swedish Cardiac Surgery Register (part of SwedeHeart). 

The Thorax Clinic at Uppsala University Hospital has been involved in valve surgery on patients with carcinoid heart disease since the late 1980s, thanks to cooperation with, and the physical proximity to, the OE clinic at Uppsala University Hospital. This is a low-frequency activity, but has increased over the last 5 years to around 4-5 patients per year, see table below. 

Table: Valve operations for carcinoid heart disease at Uppsala University Hospital 1988-2021. 

The majority of patients have come from other healthcare regions in Sweden and some have been referred internationally. Most commonly, the tricuspid and pulmonary valves have been replaced with biological valve prostheses, but occasionally patients have undergone triple or quadruple valve replacement. The surgical technique is relatively standardised and the difficulties surrounding these patients primarily involve diagnosis and selection of surgical candidates. Pre-operatively determining the best time for surgery and optimising the patient for surgery, as well as implementing perioperative care is a clinical challenge and our team at Uppsala University Hospital has gained a lot of experience in managing these patients over the years. Patients are often frail and usually require an extended stay in the thoracic ICU. Care requires good multidisciplinary collaboration, which has long been well established at the hospital with a few dedicated colleagues involved in OE, cardiology/clinical physiology, thoracic surgery and thoracic anaesthesia. In general, there is a very high level of expertise in these special patients at Uppsala University Hospital and the short-term results of the operations have improved over time and are on a par with international centres. 

Related information on other websites about neuroendocrine tumours 

Neuroendocrine system (EURACAN.eu)

ENETS Centers of Excellence (CoE) (enets.org)