Expert Consensus Practice Recommendations of the North American Neuroendocrine Tumor Society for the management of high grade gastroenteropancreatic and gynecologic neuroendocrine neoplasms

Eads, Jennifer R; Halfdanarson, Thorvardur R; Asmis, Tim; Belizzi, Andrew M; Bergsland, Emily K; Dasari, Arvind; El-Haddad, Ghassan; Frumovitz, Michael; Meyer, Joshua; Mittra, Erik; Myrehaug, Sten; Nakakura, Eric; Raj, Nitya; Soares, Heloisa P; Untch, Brian; Vijayvergia, Namrata; Chan, Jennifer A

doi:10.1530/erc-22-0206

Cited by 14 publications

(4 citation statements)

References 226 publications

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“…The stronger indication of surgery is for localized NEC, but even here, there is an indication for chemotherapy and possibly radiation (esophageal, anal some rectal tumors). The same publication states chemoradiation strategies may provide durable local control for primary and metastatic disease [ 27 ]. There is still room for discoveries and approaches regarding the best treatment.…”

Section: Discussionmentioning

confidence: 99%

Predictive Nomogram and Propensity Score Matching in Neuroendocrine Carcinoma of the Tubular Gastrointestinal Tract: A US Population-Based Clinical Outcome Study

Yasinzai,

Khan,

Chandasir

et al. 2024

Cancers

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Background: Neuroendocrine carcinomas (NECs) of the tubular gastrointestinal tract (GI-NECs) are rare and associated with worse clinical outcomes. This population-based study aims to highlight key demographics, clinicopathological factors, and survival outcomes in the US population. Methods: Data from 10,387 patients with GI-NECs were extracted from the Surveillance, Epidemiology, and End Result (SEER) database from 2000 to 2020. Results: Most patients were >40 years old at the time of presentation with a median age of 63 years old, with almost equal ethnic distribution per US population data. The most common primary tumor site was the small intestine (33.6%). The metastatic spread was localized in 34.8%, regional in 27.8%, and distant in 37.3% of cases, and the liver was the most common site of metastasis (19.9%) in known cases of metastases. Most NEC patients underwent surgery, presenting the highest 5-year overall survival of 73.2% with a 95% confidence interval (CI) (95% CI 72.0–74.4%), while chemotherapy alone had the lowest 5-year survival of 8.0% (95% CI 6.4–10.0%). Compared to men, women had a superior 5-year survival rate of 59.0% (95% CI 57.6–60.5%). On multivariate analysis, age > 65 (HR 2.49, 95% CI 2.36–2.54%, p ≤ 0.001), distant metastasis (HR 2.57, 95% CI 2.52–2.62%, p ≤ 0.001), tumor size > 4 mm (HR 1.98, 95%, CI 1.70–2.31%, p ≤ 0.001), esophageal (HR 1.49, 95% CI 0.86–2.58%, p ≤ 0.001), transverse colon (HR 1.95, 95% CI 1.15–3.33%, p ≤ 0.01), descending colon (HR 2.12, 95% CI 1.12, 3.97%, p = 0.02) anorectal sites, and liver or lung metastases were associated with worse survival. Surgical intervention and tumors located in the small intestine or appendix showed a better prognosis. Conclusion: GI-NECs are a group of rare malignancies associated with a poor prognosis. Therefore, epidemiological studies analyzing national databases may be the best alternative to have a more comprehensive understanding of this condition, assess the impact of current practices, and generate prognosis tools.

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Section: Discussionmentioning

confidence: 99%

Predictive Nomogram and Propensity Score Matching in Neuroendocrine Carcinoma of the Tubular Gastrointestinal Tract: A US Population-Based Clinical Outcome Study

Yasinzai,

Khan,

Chandasir

et al. 2024

Cancers

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“…Cisplatin or carboplatin with etoposide remains the standard first-line regimen for metastatic poorly differentiated NEC but other regimens such as FOLFIRINOX may also be reasonable. 83 The FOLFIRINEC trial (NCT04325425) is prospectively comparing cisplatin/etoposide with FOLFIRINOX as first-line therapy is ongoing. Given the highly aggressive nature of NEC, the role of surgery is very limited.…”

Section: Systemic Therapy For Necsmentioning

confidence: 99%

Consensus Guideline for the Management of Peritoneal Metastases in Patients with Neuroendocrine Neoplasms

Turaga

2024

Preprint

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Background Neuroendocrine neoplasms (NEN) with peritoneal metastases (PM) represent a complex clinical challenge due to low incidence and heterogeneous phenotypes. This manuscript describes the results of a national consensus aimed at addressing clinical management of patients with NEN-PMs. Methods An update of the 2018 Chicago consensus guidelines was conducted using a modified Delphi technique, encompassing two rounds of voting. The levels of agreement for various pathway blocks were rigorously assessed. Key systemic therapy concepts were summarized by content experts. Supporting evidence was evaluated via a rapid literature review. Results Overall, the level of evidence for the management of PM in this disease was universally low. In total, 107 participants responded in the first round, with 88/107 (82%) participating in the second round. Strong consensus (> 90%) was achieved in 5/7 (71%) and 7/7 (100%) blocks in rounds I and II respectively. A multidisciplinary approach including psychosocial and wellness assessments received a strong positive recommendation. Management of NENs with PMs was organized according to disease grade and symptom profiles. In grade 1 and 2 well-differentiated NENs, cytoreductive surgery (CRS) received strong support (>95%) following the management of functional syndromes (if present). For grade 3 well-differentiated NENs, systemic therapy is the primary recommendation, with surgical resection considered in select cases. Poorly differentiated NENs (i.e. neuroendocrine carcinomas) are predominantly treated with chemotherapy. Conclusion Given limited evidence, the consensus-driven clinical pathway offers vital clinical guidance for the management on NENs with PM. The need for high-quality evidence remains critical to the field.

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“…NANETS also recommends the use of locoregional therapies or chemotherapy for symptomatic NELM over SSAs [38,52]. For G3 NET, systematic chemotherapy is generally recommended [53]. However, NANET guidelines still support the use of liver-directed therapy for G3 NET.…”

Section: Locoregional Therapiesmentioning

confidence: 99%

Liver-Directed Locoregional Therapies for Neuroendocrine Liver Metastases: Recent Advances and Management

Criss,

Makary

2024

Current Oncology

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Neuroendocrine tumors (NETs) are a heterogeneous class of cancers, predominately occurring in the gastroenteropancreatic system, which pose a growing health concern with a significant rise in incidence over the past four decades. Emerging from neuroendocrine cells, these tumors often elicit paraneoplastic syndromes such as carcinoid syndrome, which can manifest as a constellation of symptoms significantly impacting patients’ quality of life. The prognosis of NETs is influenced by their tendency for metastasis, especially in cases involving the liver, where the estimated 5-year survival is between 20 and 40%. Although surgical resection remains the preferred curative option, challenges emerge in cases of neuroendocrine tumors with liver metastasis (NELM) with multifocal lobar involvement, and many patients may not meet the criteria for surgery. Thus, minimally invasive and non-surgical treatments, such as locoregional therapies, have surfaced. Overall, these approaches aim to prioritize symptom relief and aid in overall tumor control. This review examines locoregional therapies, encompassing catheter-driven procedures, ablative techniques, and radioembolization therapies. These interventions play a pivotal role in enhancing progression-free survival and managing hormonal symptoms, contributing to the dynamic landscape of evolving NELM treatment. This review meticulously explores each modality, presenting the current state of the literature on their utilization and efficacy in addressing NELM.

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Expert Consensus Practice Recommendations of the North American Neuroendocrine Tumor Society for the management of high grade gastroenteropancreatic and gynecologic neuroendocrine neoplasms

Cited by 14 publications

References 226 publications

Predictive Nomogram and Propensity Score Matching in Neuroendocrine Carcinoma of the Tubular Gastrointestinal Tract: A US Population-Based Clinical Outcome Study

Predictive Nomogram and Propensity Score Matching in Neuroendocrine Carcinoma of the Tubular Gastrointestinal Tract: A US Population-Based Clinical Outcome Study

Consensus Guideline for the Management of Peritoneal Metastases in Patients with Neuroendocrine Neoplasms

Liver-Directed Locoregional Therapies for Neuroendocrine Liver Metastases: Recent Advances and Management

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