Carlomagno Nicola, Santangelo Michele L, Mastromarino Rossella, Calogero Armando, Dodaro Concetta, Renda Andrea
General Surgery, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy.
General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy.
Ecancermedicalscience. 2014 Jun 18;8:438. doi: 10.3332/ecancer.2014.438. eCollection 2014.
A remarkable number of patients presents with multiple primary malignancies (MPM) over their lifetimes. In most cases inherited syndromes, iatrogenic, or viral factors are implicated, while in some cases it is not possible to ascertain a clear aetiopathogenesis.
Starting from a series of 315 patients with MPM, we focused our attention on those with extremely infrequent combinations of tumours. We retrospectively analysed patients' characteristics, type of first and second tumour and the interval between the two tumours. We made a comparison between our own data and data from surveillance, epidemiology, and end results cancer registries, the largest global series on this topic.
Six patients presented with unusual associations, namely, central nervous system (CNS)/colon, testis/stomach, colon/CNS, CNS/kidney, uterus/soft tissue, and bone/breast. The median age was 50.5 years at the diagnosis of second neoplasm and the male:female ratio was 1:1. All six patients underwent surgery for both tumours. The median interval between the first and the second tumour was 11.3 years (range 1-36 years). Five patients were given chemotherapy as adjuvant systemic treatment, and two of them with CNS tumours also received radiotherapy.
We analysed the behaviour of these rare tumours as first and second neoplasms. More frequent combinations and possible aetiological factors were evaluated.
Follow-up for patients recovering from a first tumour must be strict, as there is the risk of developing MPM, even after a long time period. Advancement in biomolecular knowledge and cooperation among different specialists are strongly needed to reduce mortality related to MPM and to foresee their occurrence.
相当数量的患者在其一生中会出现多原发性恶性肿瘤（MPM）。在大多数情况下，与遗传综合征、医源性因素或病毒因素有关，而在某些情况下，无法确定明确的病因发病机制。
从315例MPM患者系列入手，我们将注意力集中在那些肿瘤组合极为罕见的患者身上。我们回顾性分析了患者的特征、第一和第二个肿瘤的类型以及两个肿瘤之间的间隔时间。我们将自己的数据与监测、流行病学和最终结果癌症登记处的数据进行了比较，后者是关于该主题的最大全球系列数据。
6例患者出现不寻常的关联，即中枢神经系统（CNS）/结肠、睾丸/胃、结肠/CNS、CNS/肾、子宫/软组织和骨/乳腺。诊断第二个肿瘤时的中位年龄为50.5岁，男女比例为1:1。所有6例患者均接受了两种肿瘤的手术治疗。第一个和第二个肿瘤之间的中位间隔时间为11.3年（范围1 - 36年）。5例患者接受了化疗作为辅助全身治疗，其中2例患有CNS肿瘤的患者还接受了放疗。
我们分析了这些罕见肿瘤作为第一和第二个肿瘤的行为。评估了更常见的组合和可能的病因因素。
对于从第一个肿瘤康复的患者，必须进行严格随访，因为即使在很长一段时间后，仍有发生MPM的风险。迫切需要生物分子知识的进步以及不同专家之间的合作，以降低与MPM相关的死亡率并预见其发生。
