ReviewTherapeutic advances in medical oncology2020
Second-line treatment in patients with advanced extra-pulmonary poorly differentiated neuroendocrine carcinoma: a systematic review and meta-analysis.
Review in Therapeutic advances in medical oncology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
23 citing papers in PubMed, 34 citations in OpenAlex.
- Phase 2 study of cabozantinib (XL184) with nivolumab and ipilimumab for the treatment of poorly differentiated neuroendocrine carcinomas (ETCTN10315).The oncologist · 2026Trial
- Lurbinectedin plus irinotecan in pretreated gastroenteropancreatic neuroendocrine neoplasms: results of a phase II expansion cohort.ESMO open · 2026Article
- Efficacy and safety of surufatinib and sintilimab with or without chemotherapy for advanced high-grade neuroendocrine neoplasms and pancreatic cancer.NPJ precision oncology · 2026Article
- TTF-1, CDX-2, PAX-8 and GATA-3 immunoexpression in a large serie of extrapulmonary small cell neuroendocrine carcinomas: a study of 138 cases.Diagnostic pathology · 2026Article
- Progress in the treatment of esophageal neuroendocrine carcinoma (Review).Oncology letters · 2026Review
- Ramucirumab-containing chemotherapy for patients with gastrointestinal neuroendocrine carcinoma refractory/intolerant to platinum-based chemotherapy: A multicenter observational retrospective study (WJOG13420G).International journal of cancer · 2025Observational
- Clinical/pathological features and survival outcomes of extra-pulmonary neuroendocrine carcinomas: A retrospective single-center series.Journal of neuroendocrinology · 2025Article
- Correlation between p53 immunoexpression and TP53 mutation status in extrapulmonary small cell neuroendocrine carcinomas and its association with patient survival.Virchows Archiv : an international journal of pathology · 2025Article
- Prognostic factors for overall survival in advanced digestive neuroendocrine carcinoma treated with first-line cisplatin-based chemotherapy: aESMO gastrointestinal oncology · 2025Article
- Clinicopathological Characteristics of Extrapulmonary Neuroendocrine Carcinomas: Treatment Responses and Survival Outcomes: Single-Center Experience.Journal of clinical medicine · 2025Article
- Rare epithelial gastric cancers: a review of the current treatment knowledge.Therapeutic advances in medical oncology · 2025Review
- Selection of Chemotherapy in Advanced Poorly Differentiated Extra-Pulmonary Neuroendocrine Carcinoma.Cancers · 2023Review
- Managing Metastatic Extrapulmonary Neuroendocrine Carcinoma After First-Line Treatment.Current oncology reports · 2023Review
- Potent molecular-targeted therapies for gastro-entero-pancreatic neuroendocrine carcinoma.Cancer metastasis reviews · 2023Review
- NET-02: a randomised, non-comparative, phase II trial of nal-IRI/5-FU or docetaxel as second-line therapy in patients with progressive poorly differentiated extra-pulmonary neuroendocrine carcinoma.EClinicalMedicine · 2023Article
- Comparative Outcomes of Second-line Topoisomerase-I Inhibitor Therapies on Neuroendocrine Carcinoma.Journal of gastrointestinal cancer · 2023Article
- Future therapeutic strategies in the treatment of extrapulmonary neuroendocrine carcinoma: a review.Therapeutic advances in medical oncology · 2023Review
- First-line everolimus and cisplatin in patients with advanced extrapulmonary neuroendocrine carcinoma: a nationwide phase 2 single-arm clinical trial.Therapeutic advances in medical oncology · 2022Article
- Chemotherapy in NEN: still has a role?Reviews in endocrine & metabolic disorders · 2021Review
- Is the Morphological Subtype of Extra-Pulmonary Neuroendocrine Carcinoma Clinically Relevant?Cancers · 2021Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThere is no standard second-line treatment for patients with advanced extra-pulmonary poorly differentiated neuroendocrine carcinoma (EP-PD-NEC). This study explored data evaluating second-line treatment in these patients.
methodsA search of MEDLINE and EMBASE identified studies reporting survival and/or response data for patients with EP-PD-NEC receiving second-line therapy. Association between various factors (age, gender, ECOG performance status, primary tumour location, morphology, Ki-67, treatment and grade 3/4 haematological toxicity) and response rate (RR), progression-free (PFS) and overall survival (OS) were assessed with a mixed effects meta-regression weighted by individual study sample size. Due to a small sample size, associations were reported quantitatively, based on magnitude of beta coefficient rather than statistical significance.
resultsOf 83 identified studies, 19 were eligible, including 4 prospective and 15 retrospective studies. Analysis comprised 582 patients, with a median number of 19 patients in each study (range 5-100). Median age was 59 years (range 53-66). Median RR was 18% (range 0-50; 0% for single-agent everolimus, temozolomide, topotecan; 50% with amrubicin), median PFS was 2.5 months (range 1.15-6.0) and median OS was 7.64 months (range 3.2-22.0). Studies with a higher proportion of patients with a Ki-67>55% had lower RR (β = -0.73) and shorter OS (β = -0.82).
conclusionSecond-line therapy for patients with advanced EP-PD-NEC has limited efficacy and the variety of regimens used is diverse. Ki-67>55% is associated with worse outcomes. Prospective randomised studies are warranted to enable exploration of new treatment strategies.
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