To assist the oncologists in making major clinical decisions encountered while managing their patients, the Indian Council of Medical Research (ICMR) has issued draft consensus document on management of neuroendocrine cancers. Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are tumours resulting from the malignant transformation of cells in the diffuse neuroendocrine system that regulates secretion and motility in the gastrointestinal tract. The term GEP-NETs is currently the adopted nomenclature for all the NETs of the GI tract and pancreas.

This consensus statement represents the current thinking of the ICMR on the topic, based on available evidence. This document has been developed by national experts in the field and does not in any way bind a clinician to follow this guideline verbatim. Doctors can use an alternative mode of therapy on the basis of your discussions with the patient and institution and national or international guidelines.

This consensus document may be used as framework for more focused and planned research programmes to carry forward the process. The aim of this guidelines is to assist oncologists in making major clinical decisions encountered while managing their patients, while realizing the fact that some patients may require treatment strategies other than those suggested in these guidelines.

The incidence of GEP-NETs seems to be increasing globally. Some of this increased incidence may reflect better detection rates. Similar time-trends are evident in India also. Most of the prevalence and descriptive studies from India are from tertiary referral centers, with lack of population based data. The broad term GEP-NETs includes tumours in the stomach, small and large intestine, rectum and pancreas. While often considered to be rare, the incidence of this tumour is on the rise with a corresponding improvement in survival. Thus, understanding the biology and behaviour of these tumours so as to manage them appropriately, is important.

According to the consensus document, histological confirmation which includes measurement of the proliferative index (Ki67) is mandatory prior to the commencement of definitive treatment. All patients should be staged according to the TNM staging system and risk should be assessed at diagnosis. A baseline contrast-enhanced computed tomography (CECT) scan of the chest, abdomen, and pelvis should be considered. Selected cases should be referred to genetics clinics (MEN syndrome).  Patients should receive multidisciplinary care under the care of a surgical, medical, and radiation oncologist.  Primary surgery remains the standard of care for non-metastatic tumours – insulinoma, VIPoma. Patients with advanced NEC should be assessed on an individual basis to determine whether chemotherapy, targeted therapy, PRRT or best supportive care should be provided. Preferred regimens for chemotherapy include –Capecitabine-Temozolomide, Cisplatin-Etoposide and for targeted therapy – everolimus and sunitinib. Patients should be offered regular surveillance after completion of curative resection or treatment of advanced disease.