Some patients may benefit from surgery. The decision to proceed with surgery is often complex and must be tailored to each patient’s disease. Surgery may be undertaken with curative intent, to palliate symptoms, to prevent symptoms, or to prolong survival (Gangi & Anaya 2020).

When the cancer is detected early, is small and has not metastasised, it is treated with curative intent. Completely removing the entire tumour is the standard treatment, when possible. Most patients with localised NETs are successfully treated with surgery alone. If the tumour can be removed in its entirety, then surgery may cure the cancer (NECA 2019a).

The surgical procedure undertaken will depend on the location(s) of the NET and treatment intent.

It may involve different surgical specialties if disease involves more than one organ system.

Patients with NETs of the intestine may develop complications such as bowel obstruction or ischaemia. This can sometimes necessitate emergency surgery, even in the presence of unresectable metastases. Asymptomatic intestinal NETs in the presence of widespread metastases may not require any surgery. When curative intent surgery is undertaken, locoregional lymph nodes should be removed along with the GEP-NET for adequate staging and longer term disease control.

Metastases to the liver from NETs in any part of the body may potentially be managed with surgery, either with curative intent, or to improve survival if more than 70 per cent of the disease can be removed. Overall, surgery for GEP-NET is guided by the grade and degree of differentiation of the disease.

For patients with localised paraganglioma and phaeochromocytoma, surgery is usually undertaken to completely remove the tumour, and for phaeochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). Pre-operative control of blood pressure (typically with alpha-blockade) is essential for all functioning phaeochromocytomas and paragangliomas. At the time of surgery, tissue and lymph nodes will be checked for metastases, and may be removed. If both adrenal glands are removed, lifelong corticosteroid and mineralocorticoid replacement therapy will be required.

Cardiac or thoracic surgery should be considered for patients with lung NETs, metastases to the lungs, or with carcinoid heart disease who need a cardiac valve replacement.

People who have developed carcinoid syndrome are at risk of experiencing a carcinoid crisis during surgery. These patients should already be treated with somatostatin analogues (SSAs), but if not, consideration should be given to commencing them prior to surgery. To avoid major complications from a carcinoid crisis, the anaesthetic team must be fully aware of this risk before surgery so they can have treatment on hand to control the symptoms.

Intravenous octreotide is usually given before surgery to prevent carcinoid crisis. See section 6.5 for more information on carcinoid crisis.

Palliative surgery may be offered to patients when the tumour or tumours have spread or become too large to remove completely. Palliative surgery aims to ‘de-bulk’ the tumour, which could relieve some symptoms.

Timeframe for starting treatment

Timeframe for surgery will be based on investigation and staging of the NET and surgery intent.

Training and experience required of the surgeon

Fellow of the Royal Australian College of Surgeons (or equivalent) with adequate training and experience that enables institutional credentialing and agreed scope of practice in this area.

Documented evidence of the surgeon’s training and experience, including their specific (sub-specialty) experience with NETs and procedures to be undertaken, should be available.

Different surgeons may be involved such as colorectal, hepatobiliary, and cardiothoracic.

There is strong evidence to suggest that institutions with a high volume of NET resections have better clinical outcomes for complex cancer surgery (Toomey et al. 2016).

Health service characteristics

To provide safe and quality care for patients having surgery, health services should have these features:

• critical care support
• 24-hour medical staff availability
• 24-hour operating room access and intensive care unit
• diagnostic imaging
• pathology
• nuclear medicine imaging.