Massironi S, Campana D, Pusceddu S, Albertelli M, Faggiano A, Panzuto F, Smiroldo V, Andreasi V, Rossi R E, Maggio I, Torchio M, Dotto A, Modica R, Rinzivillo M, Carnaghi C, Partelli S, Fanetti I, Lamberti G, Corti F, Ferone D, Colao A, Annibale B, Invernizzi P, Falconi M
Division Gastroenterology, San Gerardo Hospital, University of Milano - Bicocca School of Medicine, Via Pergolesi 33, 20900 Monza (MB), Italy.
NET Team Bologna, ENETS Center of Excellence, "S. Orsola-Malpighi" University Hospital, "Alma Mater Studiorum" University of Bologna, Bologna, Italy.
Dig Liver Dis. 2021 Mar;53(3):367-374. doi: 10.1016/j.dld.2020.09.031. Epub 2020 Oct 22.
Patients with sporadic neuroendocrine neoplasms may exhibit a higher risk of a second primary tumor than the general population.
This study aimed to analyze the occurrence of second primary malignancies.
A retrospective cohort of 2757 patients with sporadic lung and gastro-entero-pancreatic neuroendocrine neoplasms, managed at eight Italian tertiary referral Centers, was included.
Between 2000 and 2019, a second primary malignancy was observed in 271 (9.8%) neuroendocrine neoplasms patients with 32 developing a third tumor. There were 135 (49.8%) females and the median age was 64 years. The most frequent locations of the second tumors were breast (18.8%), prostate (12.5%), colon (9.6%), blood tumors (8.5%), and lung (7.7%). The second primary tumor was synchronous in 19.2% of cases, metachronous in 43.2%, and previous in 37.6%. As concerned the neuroendocrine neoplasms, the 5- and 10-year survival rates were 87.8% and 74.4%, respectively. PFS for patients with a second primary malignancy was shorter than for patients without a second primary malignancy. Death was mainly related to neuroendocrine neoplasms.
In NEN patients the prevalence of second primary malignancies was not negligible, suggesting a possible neoplastic susceptibility. Overall survival was not affected by the occurrence of a second primary malignancy.
散发性神经内分泌肿瘤患者发生第二原发性肿瘤的风险可能高于普通人群。
本研究旨在分析第二原发性恶性肿瘤的发生情况。
纳入了在意大利8个三级转诊中心接受治疗的2757例散发性肺和胃肠胰神经内分泌肿瘤患者的回顾性队列。
2000年至2019年期间，在271例（9.8%）神经内分泌肿瘤患者中观察到第二原发性恶性肿瘤，其中32例发生了第三种肿瘤。女性有135例（49.8%），中位年龄为64岁。第二原发性肿瘤最常见的部位是乳腺（18.8%）、前列腺（12.5%）、结肠（9.6%）、血液肿瘤（8.5%）和肺（7.7%）。第二原发性肿瘤在19.2%的病例中为同步性，43.2%为异时性，37.6%为先前存在性。就神经内分泌肿瘤而言，5年和10年生存率分别为87.8%和74.4%。有第二原发性恶性肿瘤的患者的无进展生存期短于无第二原发性恶性肿瘤的患者。死亡主要与神经内分泌肿瘤有关。
在神经内分泌肿瘤患者中，第二原发性恶性肿瘤的患病率不可忽视，提示可能存在肿瘤易感性。第二原发性恶性肿瘤的发生并未影响总生存期。
