Ann Intern Med. 2015 May 19;162(10):682-9. doi: 10.7326/M14-2132.
Pancreatic neuroendocrine tumors (PNETs) may evolve and cause hormonal hypersecretion-related symptoms that were not present at the initial diagnosis, termed metachronous hormonal syndromes (MHSs). Their setting, characteristics, and outcomes are not well-described.
To describe MHSs in patients with sporadic PNETs.
Retrospective, multicenter study.
4 French referral centers.
Patients with PNETs who developed MHSs related to hypersecretion of insulin, gastrin, vasoactive intestinal peptide, or glucagon between January 2009 and January 2014.
Tumor extension, biological markers, and treatments at initial PNET diagnosis and MHS onset. Pathologic specimens were evaluated centrally, including Ki-67 index and hormone immunolabeling.
Of 435 patients with PNETs, 15 (3.4%) were identified as having MHSs involving the hypersecretion of insulin (5 patients), vasoactive intestinal peptide (5 patients), gastrin (2 patients), or glucagon (4 patients). Metachronous hormonal syndromes developed after a median of 55 months (range, 7 to 219) and in the context of PNET progression, stability, and tumor response in 8, 6, and 1 patients, respectively. The median Ki-67 index was 7% (range, 1% to 19%) at PNET diagnosis and 17.5% (range, 2.0% to 70.0%) at MHS onset. Immunolabeling of MHS-related peptides was retrospectively found in 8 of 14 of pathologic PNET specimens obtained before MHS diagnosis. Median survival after MHS onset was 28 months (range, 3 to 56). Seven patients with MHSs died during follow-up, all due to PNETs, including 4 patients with insulin-related MHSs.
Retrospective data collection and heterogeneity of pathologic specimen size and origin.
Metachronous hormonal syndromes were identified more often in the context of PNET progression and increased Ki-67 indices. Patients with insulin-related MHSs may have decreased survival rates.
None.
胰腺神经内分泌肿瘤（PNETs）可能会发展并引起初始诊断时不存在的激素过度分泌相关症状，称为同步激素综合征（MHSs）。它们的发生、特征和结局尚不清楚。
描述散发性 PNET 患者的 MHSs。
回顾性、多中心研究。
法国 4 个转诊中心。
2009 年 1 月至 2014 年 1 月期间，PNET 患者发生胰岛素、胃泌素、血管活性肠肽或胰高血糖素过度分泌相关 MHSs。
初始 PNET 诊断和 MHS 发病时的肿瘤扩展、生物标志物和治疗。中心评估病理标本，包括 Ki-67 指数和激素免疫标记。
在 435 例 PNET 患者中，有 15 例（3.4%）被确定为发生 MHSs，涉及胰岛素（5 例）、血管活性肠肽（5 例）、胃泌素（2 例）或胰高血糖素（4 例）过度分泌。MHSs 中位发病时间为 55 个月（范围，7 至 219），分别在 8、6 和 1 例患者中处于 PNET 进展、稳定和肿瘤反应背景下。初始 PNET 诊断时的 Ki-67 指数中位数为 7%（范围，1%至 19%），MHS 发病时为 17.5%（范围，2.0%至 70.0%）。在 MHS 诊断前获得的 14 份病理 PNET 标本中，有 8 份回顾性发现与 MHS 相关肽的免疫标记。MHS 发病后中位生存期为 28 个月（范围，3 至 56）。7 例 MHSs 患者在随访期间死亡，均死于 PNET，其中 4 例为胰岛素相关 MHSs。
回顾性数据收集以及病理标本大小和来源的异质性。
MHSs 更常发生在 PNET 进展和 Ki-67 指数增加的背景下。胰岛素相关 MHSs 患者的生存率可能较低。
无。
