European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for Digestive Neuroendocrine Carcinoma

This ENETS guidance paper, developed by a multidisciplinary working group, provides up-to-date and practical advice on the diagnosis and management of digestive neuroendocrine carcinoma, based on recent developments and study results. These recommendations aim to pave the road for more standardized care for our patients resulting in improved outcomes. However, it’s true to say that the prognosis for differentiated Neuroendocrine Carcinomas (NEC) is generally poorer than the less aggressive Neuroendocrine Tumours (NET).

Surgery can be of benefit for localized disease after extensive preoperative imaging. Carboplatin in combination with etoposide is recommended as first-line treatment for metastatic disease. Irinotecan with fluoropyrimidines has the best evidence as second-line treatment. Immunotherapy plays a minor role in biomarker-unselected patients. Molecular profiling if available is encouraged to identify new targets. More prospective clinical trials are highly needed to fulfil the unmet needs in this field, especially on new predictive and prognostic biomarkers and to improve survival of patients with advanced disease.

Given the naming of the WHO Classification of Tumours publications (pending the forthcoming 2022 wide scope publication), the term “digestive” might be confusing to some but I can confirm it includes the gastrointestinal tract plus pancreatic NECs effectively making it a Gastroenteropancreatic (GEP) NEC guidance document.

TABLE 1. Ten major questions on clinical, pathological as well as molecular diagnostics, prognostic factors and management of digestive neuroendocrine carcinoma (NEC).

Q1 – What clinical examinations for diagnosis and staging should be performed in a newly diagnosed digestive NEC?

Q2 – What pathological workup is needed for a newly diagnosed digestive NEC?

Q3 – What are the prognosis and prognostic factors for digestive NEC?

Q4 – How should surgery, chemoradiation and neoadjuvant chemotherapy be used in localized (stage I–III) digestive NEC?

Q5 – Should adjuvant chemotherapy be given for localized (stage I–III) digestive NEC?

Q6 – What should be given as first-line treatment for metastatic digestive NEC?

Q7 – What should be given as second- and third-line treatment for metastatic digestive NEC

Q8 – Should immunotherapy (immune checkpoint inhibitors) be used for treatment of digestive NEC?

Q9 – Is molecular/genetic based therapy an option for digestive NEC?

Q10 – How should high-grade digestive MiNEN be diagnosed and treated?

Now read the reference material on Digestive Neuroendocrine Carcinoma below

Reference

Click on the blue link below see read the discussion and the recommendations for each of those questions.

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma

Sorbye H, Grande E, Pavel M, Tesselaar M, Fazio N, Reed NS, Knigge U, Christ E, Ambrosini V, Couvelard A, Tiensuu Janson E. European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma. J Neuroendocrinol. 2023 Mar;35(3):e13249. doi: 10.1111/jne.13249. Epub 2023 Mar 16. PMID: 36924180.

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