China Anti-Cancer Association guideline for diagnosis and treatment of neuroendocrine neoplasms (2025 edition)

Abstract

Neuroendocrine neoplasms (NENs) are a rare group of tumors that originated from peptidergic neurons and neuroendocrine cells, which has neuroendocrine differentiation characteristics and expresses neuroendocrine markers. NENs occur in all parts of the body, especially in lung, gastrointestinal tract and pancreas. Both domestic and foreign researches showed that the incidence of NENs is on the rise. NENs have high heterogeneity and can originate in multiple tissues and organs, including pituitary, thyroid, parathyroid, skin, bronchial lung and thymus, gastrointestinal tract and pancreas, adrenal glands, genitourinary organs, etc. At the same time, when the classification and grading of NENs originating from the same tissue or organ are different, they also have significantly different biological behaviors. The high heterogeneity of NENs determines the difficulty and complexity of its diagnosis. In addition to clinical symptoms, it also needs to include special biomarkers, endoscopy, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and various functional imaging methods for integrated diagnosis. In addition, the treatment of NENs also covers endoscopic therapy, surgical treatment, interventional therapy, drug therapy, radiotherapy and peptide receptor radionuclide therapy (PRRT). The development of treatment strategies requires not only following guidelines, but also making individual choices on the basis of multidisciplinary team (MDT) collaboration and integrated diagnosis and treatment. In 2024, the Society of Neuroendocrine Neoplasm of China Anti-Cancer Association once again organized experts in relevant fields to formulate the “China Anti-Cancer Association guideline for diagnosis and treatment of neuroendocrine neoplasms (2025 edition)” on the basis of the “China Anti-Cancer Association guideline for diagnosis and treatment of neuroendocrine neoplasm (2022 edition)”, domestic and international guidelines and consensus, as well as the latest clinical research results. The guideline had been registered on Practice guideline REgistration for transPAREncy (PREPARE) with the registration number PREPARE-2024CN1158. The 2025 edition updated and revised the relevant content from the 2022 edition and further expanded recommendations beyond thoracic, gastrointestinal tract and pancreas NENs, including pituitary neuroendocrine tumors (PitNETs), medullary thyroid carcinoma (MTC), pheochromocytoma/paragangliomas (PPGLs) and Merkel cell carcinoma (MCC), with the expectation of providing references for clinical practitioners.

Keywords

• |neuroendocrine neoplasms|diagnosis|treatment|guideline