Neuroendocrine neoplasms (NENs) represent a rare and heterogenous group of tumors with predominantly gastroenteropancreatic or pulmonary origin. Despite numerous diagnostic efforts, the primary tumor site remains unknown in up to 20% of the patients diagnosed with NEN. In this subgroup of NEN patients, a standard diagnostic algorithm has not yet been integrated into clinical routine. Of note, an undetermined primary tumor site in NENs is associated with an impaired clinical outcome by at least “formally” limiting treatment options exclusively approved for NENs of a certain histological origin. In this retrospective study, a patient cohort of 113 patients initially diagnosed with NEN of unknown primary (NEN-UP) was analyzed. In 13 patients (11.5%) a primary tumor site could be identified subsequently, amongst others, by performing somatostatin receptor (SSTR)-PET-based imaging, which was irrespective of the initial clinical or demographic features. Diagnostic work-up and therapeutic regimens did not differ significantly between patients with an identified or unidentified primary tumor site; only a detailed immunohistochemical assessment providing additional information on the tumor origin proved to be significantly associated with the detection of a primary tumor site. Our study revealed that a profound diagnostic work-up, particularly including SSTR-PET-based imaging, leads to additional treatment options, finally resulting in significantly improved clinical outcomes for patients with NEN-UPs.
神经内分泌肿瘤（NENs）是一组罕见且异质性强的肿瘤，主要起源于胃肠胰腺或肺部。尽管进行了大量诊断努力，仍有高达20%的NEN患者在确诊时原发肿瘤部位不明。对于这一NEN亚群患者，目前尚未将标准诊断流程纳入临床常规。值得注意的是，NEN原发部位未明确会限制仅针对特定组织学起源NEN的获批治疗方案，从而“形式上”至少导致临床预后受损。本回顾性研究分析了113例最初诊断为原发灶不明神经内分泌肿瘤（NEN-UP）的患者队列。其中13例患者（11.5%）通过生长抑素受体（SSTR）-PET影像学检查等方法最终确定了原发肿瘤部位，且该结果与初始临床或人口学特征无关。原发灶明确与未明确患者间的诊断检查方案和治疗方案无显著差异；仅能提供肿瘤起源额外信息的详细免疫组化评估被证实与原发灶的检出显著相关。我们的研究表明，深入的诊断检查（特别是包含SSTR-PET影像学检查）能为NEN-UP患者带来更多治疗选择，最终显著改善其临床预后。
肿瘤(癌症)患者之家