Provincial Health Services Authority (PHSA) improves the health of British Columbians by seeking province-wide solutions to specialized health care needs in collaboration with BC health authorities and other partners.
Revised 27 November 2018
General Aspects - Introduction
From a clinical standpoint, NETs can be divided into two groups: functioning and nonfunctioning. Functioning NETs hypersecrete hormones that cause specific syndromes (e.g. carcinoid syndrome) and they are named according to the hypersecreted hormone (insulinoma, gastrinoma, etc). The symptoms caused by the hypersecretion of these hormones often lead to their discovery. Nonfunctioning tumours, which account for about one-third to one-half of NETs, are not associated with a hypersecreted-related clinical syndrome. They come to attention because of their “mass effect” due to tumour bulk. Metastatic disease is often present at diagnosis. Because they are usually slow growing, NETs are frequently diagnosed late in their course. Those arising in the gut can cause intermittent abdominal discomfort for months or years, often interpreted to be a functional disorder. Later, bowel obstruction occurs secondary to desmoplastic reaction of the mesentery or, less commonly, from the tumour.
A correct histological diagnosis is critical and this requires an adequate biopsy. A distinction should be made between a well-differentiated and a poorly differentiated neoplasm as well as between well differentiated benign endocrine neoplasms, neoplasms of uncertain behavior and malignant neoplasms. This distinction can be aided by several features of the tumour: size, invasion of adjacent tissue or wall, invasion beyond the submucosa, angioinvasion, perineural invasion, a solid organoid structure, presence of necrosis, mitoses per high power field, Ki67 index, loss of chromogranin A immunoreactivity or hormone expression.
Radiologic studies and nuclear imaging play an important role in the diagnosis and management of patients with NETs.
Definitive management includes tumour resection for cure. When this is not achievable, the goals of treatment include symptom control, biochemical control (i.e. controlling excess bioactive peptides), objective tumour control and improving patient quality of life. In recent years, the management has become complex with the introduction of a number of new strategies; hence, a multidisciplinary approach is recommended.
BC Cancer Referral Process for Peptide Receptor Radionuclide Therapy (PRRT) using Lutetium 177Lu-Dotatate (Lutathera) for Treatment in Patients with Somatostatin Receptor Positive Midgut Neuroendocrine Tumors
Updated: March 8, 2022
At this time, PRRT is only available at BC Cancer – Vancouver Centre. Please see the UGIPRRT protocol for details of patient eligibility. If you have a patient that meets these criteria and is interested in able/willing to travel to Vancouver for treatment, we have outlined below the steps for referral.
Prior to referral, please have the following investigations completed:
After these investigations are in place, please refer the case to the provincial Tuesday morning GI multidisciplinary tumor board for review and approval. After approval by the tumor board, a conference note should be dictated and placed on the chart and approval for funding of the UGIPRRT protocol should be performed through the BC Cancer Compassionate Access Program (CAP) by the referring provider. After these have been approved, the Vancouver PRRT team will arrange an in person consultation and further therapy.
During PRRT, patients will be reviewed by the treating team in Vancouver, however treating oncologists at other sites are requested to continuing managing long acting somatostatin analogue prescriptions and supportive medications and to facilitate a transfer of care after completion of PRRT. For any questions, please contact Dr. Jonathan Loree (Medical Oncology) or Dr. Don Wilson (Nuclear Medicine).
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