Indian Council of Medical Research Consensus Document for the Management of Gastroenteropancreatic Neuroendocrine Neoplasms

Patients with advanced gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) should be assessed on an individual basis to determine whether chemotherapy, targeted therapy, PRRT, or best supportive care should be providedPreferred regimens for chemotherapy include – capecitabine-temozolomide, cisplatin-etoposide and for targeted therapy – everolimus and sunitinibPatients should be offered regular surveillance after completion of curative resection or treatment of advanced diseaseEncourage participation in institutional and ethical review board-approved, registered controlled clinical trialsRefer for early palliative care, if indicated. [7] Purpose Although international guidelines are available for the management of GEP-NENs, it is not entirely feasible to apply these guidelines to the Indian population owing to differences in the incidence of the disease in different parts of India, varying socioeconomic factors, and availability of resources. [...]it is essential to analyze the evidence pertaining to GEP-NENs from India and the rest of the world[13] with an aim to formulate evidence-based guidelines that could be applicable to Indian patients. Grading of gastroenteropancreatic neuroendocrine neoplasms Immunohistochemistry (IHC) estimation of the Ki-67 expression and mitotic index count are used to grade GEP-NENs [Table 1], as per the World Health Organization (WHO) classification [Table 2], into low-grade (G1), intermediate-grade (G2), and high-grade (G3) categories. [...]since the emerging evidence suggested that the G3 pancreatic NEN are heterogeneous in morphology and biology, the recent WHO 2017 classification has introduced a new category of well-differentiated pancreatic neuroendocrine tumors (WD-pNETs) G3, that show lower response rate to platinum-based chemotherapy while better outcomes compared with poorly differentiated pancreatic NECs (PD-pNECs) G3.