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* For healthcare professionals to read reference, please do not forward Preface In recent years the concept of diagnosis and treatment of neuroendocrine tumors and treatment strategies have a larger update, increasingly rich content
.
In order to better guide clinical practice, experts from the Department of Pathology, Department of Oncology, and Department of Imaging have participated in the compilation of part of the guide, reflecting the progress of comprehensive diagnosis and treatment of neuroendocrine tumors from various professional perspectives
.
The guidelines cover chapters on the principles of diagnosis and treatment of neuroendocrine tumors, diagnostic methods, treatment methods, follow-up, and genetic related content of neuroendocrine tumors, and increase the scientific and authoritative nature of the guidelines, so as to better provide references for clinical diagnosis and treatment strategies and path selection.
Guidance
.
Neuroendocrine tumors (NENs) have always been considered a rare tumor, but the incidence of NENs in China has been on the rise in recent years
.
NENs can originate in various parts of the body and are highly heterogeneous
.
According to the degree of differentiation, it is divided into well-differentiated and slow-growing neuroendocrine tumors (NETs) and poorly differentiated and highly malignant neuroendocrine carcinomas (NEC); tumors are divided into functional and non-functional tumors according to whether they secrete peptide hormones
.
The symptoms and signs of NENs patients are different, and it is easy to be misdiagnosed in the clinic.
Most patients are in the advanced stage when they are diagnosed, and only a few patients have the opportunity to undergo radical surgical resection
.
At the same time, due to the heterogeneity of NENs and individual differences in patients, there is much uncertainty between clinical diagnosis and treatment behavior and outcomes
.
Therefore, the diagnosis and treatment of NENs should adopt the MDT model to facilitate accurate diagnosis and tailor individualized treatment strategies for patients to optimize patient benefits
.
MDT mode assists accurate diagnosis of NENs.
Standard clinical diagnosis requires pathological diagnosis, image positioning and diagnosis, and biochemical diagnosis: • Pathological graded diagnosis is the key to the diagnosis of NENs
.
Tumors can be classified and graded through pathological diagnosis
.
• Imaging examination: It is of great significance to the clinical staging of tumors
.
At the same time, it has an important prompting role for tumor localization and qualitative diagnosis and qualitative diagnosis has a highly prompting role
.
In addition to traditional ultrasound, CT, MRI, 18F-FDG PET/CT and other examinations, somatostatin receptor imaging has become an important examination method for the diagnosis of NENs
.
• The diagnosis of functional NENs includes qualitative diagnosis and localized diagnosis.
In addition to clinical symptoms, the detection of different biochemical indicators is the main basis for diagnosis
.
The MDT model provides individualized treatment for patients with NENs.
The treatment of non-metastatic NENs is mainly surgery and endoscopic resection
.
If local resection cannot be performed due to various reasons, it is recommended to perform comprehensive treatment based on systemic treatment under the guidance of MDT.
If it is transformed into a resectable tumor, surgical operation can be actively performed
.
For postoperative adjuvant treatment, a number of retrospective analyses have shown that limited-period surgery for gastrointestinal NEC can benefit from postoperative adjuvant treatment, but there is no high-level evidence to confirm the efficacy of postoperative adjuvant treatment for NETs
.
One of the important treatments for metastatic NETs is local treatment, which is recommended after discussion by MDT
.
In the case of simple liver metastasis, according to the different types of liver metastases from ENETS, radical resection of the primary tumor/metastasis, or local ablation, transhepatic artery embolization (TAE), etc.
can be selected; if extrahepatic metastasis occurs, systemic therapy is recommended Comprehensive treatment based on systemic treatment, if necessary, treatment will be carried out after discussion by MDT
.
For patients who cannot be cured by surgery, systemic systemic treatment is indispensable.
The purpose of systemic treatment includes anti-tumor proliferation and control of hormone-related symptoms.
It can be combined with local treatments such as surgery, TAE or radiofrequency ablation to reduce tumor burden, especially It is for functional tumors
.
Depending on the location of the primary tumor, the choice of therapeutic drugs is also different, including somatostatin analogs (SSAs) (such as lanreotide and octreotide), molecular targeted drugs (such as everolimus, sofantinib) , chemotherapy, receptor-mediated peptide radionuclide therapy (PRRT) and the like
.
NEC is relatively rare, including small cell and large cell types, and can occur in a variety of organs, or the primary focus can be unknown
.
Similar to the characteristics of high invasion and metastasis of small cell lung cancer, most NECs are staged late at the time of diagnosis or have been accompanied by distant metastasis, and the prognosis is poor
.
For locally advanced, unresectable or metastatic NEC, cisplatin or carboplatin combined with etoposide is recommended as the first-line treatment
.
At present, the second-line treatment lacks a standard plan.
For patients with metastatic NEC who have received systemic treatment in the past and continue to progress, immune checkpoint inhibitor therapy can also be considered
.
For the follow-up of NENs patients, lifelong follow-up is recommended for patients with NENs except for patients with NETs originating from the appendix or rectum G1 and having a maximum diameter of less than 1 cm.
Long-term follow-up is not required after R0 resection
.
Follow-up of all patients includes clinical examination (the control of tumors and functional syndromes need to be evaluated) and routine imaging examinations
.
Genetic syndrome-related NENs.
About 5% of NENs occur and develop closely related to genetic factors.
They have clear gene mutations or deletions and even chromosomal changes.
They are usually inherited in an autosomal dominant manner, and the clinical manifestations include multiple NENs.
Tumor syndromes, with various clinical manifestations, are called genetic syndrome-related NENs, such as multiple endocrine neoplasia type 1 (MEN1) and type 2 (MEN2), tuberous sclerosis (TSC), etc.
.
Patients with genetic risks need to be screened and genetically tested
.
Reference: CSCO Neuroendocrine Tumor Tumor Diagnosis and Treatment Guidelines 2021 Approval Number: NP-STD-21N92-Valid Until 2023 Oct