Alexandraki Krystallenia I, Tsoli Marina, Kyriakopoulos Georgios, Angelousi Anna, Nikolopoulos Georgios, Kolomodi Denise, Kaltsas Gregory A
Unit of Endocrinology, First Department of Internal Medicine, Laiko University Hospital, National and Kapodistrian University of Athens, Athens, Greece -
Unit of Endocrinology, First Department of Internal Medicine, Laiko University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Minerva Endocrinol. 2019 Dec;44(4):378-386. doi: 10.23736/S0391-1977.19.03012-8. Epub 2019 Apr 15.
Neuroendocrine neoplasms (NENs) of unknown primary origin (UPO-NENs) are advanced neoplasms constituting 11-22% of all NENs that by definition their primary tissue of origin has not been identified with standard diagnostic work-up. Delineating the primary site of origin of UPO-NENs has important implications for selecting the appropriate treatment and overall prognosis. The small bowel, followed by the lung and pancreas are the most prevalent primary sites of origin of UPO-NENs that are uncovered during an extensive and prolonged diagnostic work-up; however, a number of UPO-NENs may still remain occult even after prolonged follow-up. A number of diagnostic algorithms that incorporate histopathological, molecular, imaging (either morphological or functional imaging), and serum biomarkers can help to identify the primary tumor origin. It is expected that advances in these fields will help reduce significantly the number of UPO-NENs.
原发灶不明的神经内分泌肿瘤（UPO-NENs）是晚期肿瘤，占所有神经内分泌肿瘤的11%-22%，根据定义，其原发组织来源尚未通过标准诊断检查确定。明确UPO-NENs的原发部位对于选择合适的治疗方法和总体预后具有重要意义。小肠是UPO-NENs最常见的原发部位，其次是肺和胰腺，这些原发部位是在广泛而长期的诊断检查中发现的；然而，即使经过长期随访，仍有一些UPO-NENs可能仍隐匿未被发现。一些结合组织病理学、分子学、影像学（形态学或功能成像）和血清生物标志物的诊断算法有助于识别原发肿瘤的起源。预计这些领域的进展将有助于显著减少UPO-NENs的数量。
