Scholarlink

<p>EC-cell familial small intestine neuroendocrine tumors (EC-cell F-SINET) are a recently described but poorly characterized entity. We aimed to describe their clinical and pathological features, and to compare them to patients with a sporadic form (EC-cell S-SINET). We constituted a nationwide cohort including (retrospectively patients diagnosed before 2012 and prospectively from 2012 to 2022) all patients with F-SINET (histologically proven SINET in ≥ 2 first or second-degree relatives) managed in the French GTE-RENATEN network. Clinical and pathological data were described and compared to the GTE-RENATEN population-based cohort including 2460 patients with EC-cell S-SINET using multivariable logistic regression. The survival impact of EC-cell F-SINET was explored using Cox proportional hazard analyses. We included 92 patients with EC-cell F-SINET from 47 families. Median age at diagnosis was 60.4 years. Among these patients, 22% had a history of another cancer, 76.1% had synchronous or metachronous metastases, 38% had a carcinoid tumor syndrome, and median Ki-67 labeling index was 2%. Median survival was 204.6 months. Multifocal EC-cell SINET were present in 66.7% of patients with EC-cell F-SINET and were associated with poorer prognosis (<i>p</i> = 0.005). In comparison with EC-cell S-SINET patients, those with EC-cell F-SINET had more frequent multifocal primary tumor in the small bowel (<i>p</i> &lt; 0.0001) and carcinoid syndrome (<i>p</i> = 0.038), lower Ki-67 index and tended to be younger (<i>p</i> = 0.094). After adjustment on independent prognostic factors (age at diagnosis, metastatic stage, carcinoid tumor syndrome and Ki-67 index), EC-cell F-SINET did not have a different prognosis than that of EC-cell S-SINET. Patients with EC-cell F-SINET are characterized by more frequent multifocal EC-cell SINETs and more frequent carcinoid tumor syndrome, but their prognosis seems similar compared to patients with EC-cell S-SINET.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Clinical and Pathological Features of EC-cell Familial Small Intestine Neuroendocrine Tumors: A Nationwide Cohort From the French GTE-RENATEN Network

  • 期刊论文

  • 发表日期：

    2026年3月19日

查看全文
  • Thomas Hunaut,
  • Laura Gérard,
  • Agathe Hercent,
  • Olivia Hentic,
  • Bruno Buecher,
  • Come Lepage,
  • Thierry Lecomte,
  • Philippe Thuillier,
  • Christine Do Cao,
  • Alice Durand,
  • Guillaume Roquin,
  • Céline Lepere,
  • Maelle Lebras,
  • Sophie Dominguez,
  • Philippe Baltzinger,
  • Pierre Devulder,
  • Nathalie Guedj,
  • Hedia Brixi,
  • Thomas Féron,
  • Jérôme Cros,
  • Eric Pasmant,
  • Catherine Lombard-Bohas,
  • Thomas Walter,
  • Guillaume Cadiot,
  • Louis de Mestier

摘要

EC-cell familial small intestine neuroendocrine tumors (EC-cell F-SINET) are a recently described but poorly characterized entity. We aimed to describe their clinical and pathological features, and to compare them to patients with a sporadic form (EC-cell S-SINET). We constituted a nationwide cohort including (retrospectively patients diagnosed before 2012 and prospectively from 2012 to 2022) all patients with F-SINET (histologically proven SINET in ≥ 2 first or second-degree relatives) managed in the French GTE-RENATEN network. Clinical and pathological data were described and compared to the GTE-RENATEN population-based cohort including 2460 patients with EC-cell S-SINET using multivariable logistic regression. The survival impact of EC-cell F-SINET was explored using Cox proportional hazard analyses. We included 92 patients with EC-cell F-SINET from 47 families. Median age at diagnosis was 60.4 years. Among these patients, 22% had a history of another cancer, 76.1% had synchronous or metachronous metastases, 38% had a carcinoid tumor syndrome, and median Ki-67 labeling index was 2%. Median survival was 204.6 months. Multifocal EC-cell SINET were present in 66.7% of patients with EC-cell F-SINET and were associated with poorer prognosis (p = 0.005). In comparison with EC-cell S-SINET patients, those with EC-cell F-SINET had more frequent multifocal primary tumor in the small bowel (p < 0.0001) and carcinoid syndrome (p = 0.038), lower Ki-67 index and tended to be younger (p = 0.094). After adjustment on independent prognostic factors (age at diagnosis, metastatic stage, carcinoid tumor syndrome and Ki-67 index), EC-cell F-SINET did not have a different prognosis than that of EC-cell S-SINET. Patients with EC-cell F-SINET are characterized by more frequent multifocal EC-cell SINETs and more frequent carcinoid tumor syndrome, but their prognosis seems similar compared to patients with EC-cell S-SINET.