Domati Federica, Maffei Stefania, Kaleci Shaniko, Di Gregorio Carmela, Pedroni Monica, Roncucci Luca, Benatti Piero, Magnani Giulia, Marcheselli Luigi, Bonetti Luca Reggiani, Mariani Francesco, Alberti Antonio Maria, Rossi Valerio, Ponz de Leon Maurizio
Dipartimento di Medicina Diagnostica, Clinica e Sanità Pubblica, Università di Modena e Reggio, Emilia, Italy.
Intern Emerg Med. 2014 Sep;9(6):623-31. doi: 10.1007/s11739-013-0981-3. Epub 2013 Aug 9.
The aim of the study was to investigate the clinical features, including survival, of patients with colorectal malignancies developed at a very early age (≤40 years), together with possible factors involved in the pathogenesis of these rare neoplasms. The study took advantage of the existence of a specialized colorectal cancer Registry active from 1984. 57 patients met the criteria of early onset cancer; main epidemiological data, morphology, stage, familial aggregation, possible role of inheritance and survival were analyzed. Despite the relevant increase over time of all registered patients, joiningpoint analysis of crude incidence rate of early onset colorectal neoplasms revealed a certain stability of these tumors (EAPC: 2.4, CI 14-22) with a constant prevalence of the male sex. Stage at diagnosis did not show significant variations between early onset and maturity onset colorectal neoplasms. Hereditary as well as familial cases were significantly (P < 0.005 and 0.03) more frequent among patients with early onset tumors, although in the majority of them no specific etiological factor could be identified. Survival was more favorable in patients with early onset tumors, though this had to be attributed to the higher presence of some histological types in early onset cases. Survival was significantly more favorable for patients of all ages registered in the last decade. Incidence of early onset colorectal cancer was relatively stable between 1984 and 2008. A male preponderance was evident through the registration period. Hereditary and familial cases were significantly more frequent among early onset case. A well defined etiology could be observed in 16% of the cases (versus 2-3% in older individuals). Five-year survival showed a significant improvement over time.
本研究旨在调查极早期(≤40岁)发生结直肠恶性肿瘤患者的临床特征,包括生存率,以及这些罕见肿瘤发病机制中可能涉及的因素。该研究利用了自1984年起活跃的专门结直肠癌登记处。57例患者符合早发性癌症标准;分析了主要流行病学数据、形态学、分期、家族聚集性、遗传的可能作用及生存率。尽管所有登记患者的数量随时间显著增加,但早发性结直肠肿瘤粗发病率的连接点分析显示这些肿瘤具有一定稳定性(EAPC:2.4,CI 14 - 22),且男性患病率恒定。早发性和成年期发生的结直肠肿瘤在诊断时的分期无显著差异。早发性肿瘤患者中遗传性及家族性病例显著更常见(P < 0.005和0.03),尽管其中大多数无法确定具体病因。早发性肿瘤患者的生存率更优,不过这归因于早发性病例中某些组织学类型的占比更高。过去十年登记的各年龄段患者生存率显著更优。1984年至2008年间,早发性结直肠癌发病率相对稳定。在整个登记期间,男性占优势明显。早发性病例中遗传性和家族性病例显著更常见。16%的病例可观察到明确病因(老年个体为2 - 3%)。五年生存率随时间有显著改善。