Raciborska Anna, Bilska Katarzyna, Węcławek-Tompol Jadwiga, Ussowicz Marek, Pogorzała Monika, Janowska Joanna, Rychłowska-Pruszyńska Magdalena, Rodriguez-Galindo Carlos, Helwich Ewa
Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland.
Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland.
Pediatr Neonatol. 2016 Aug;57(4):295-301. doi: 10.1016/j.pedneo.2015.08.007. Epub 2015 Dec 1.
Advances in multidisciplinary care for pediatric cancer have resulted in significant improvement in cure rates over the last decades; however, these advances have not been uniform across all age groups. Cancer is an important cause of perinatal mortality, yet the full spectrum of malignant neoplasms in newborns is not well defined.
The authors have reviewed the clinical features and outcomes of 37 newborns with congenital malignant tumors treated at three referral centers in North, Central, and South Poland between 1980 and 2014. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier methods and compared using long-rank test and Cox models.
Twenty-two patients were diagnosed prenatally. The most common diagnoses were neuroblastoma (48.7%), followed by malignant germ-cell tumor (16.2%), and Wilms' tumor (8.1%). Neuroblastoma was the most common malignancy among full-term infants, and malignant sacrococcygeal teratoma was the most common malignancy in premature infants. Thirty patients (81%) are alive with a median follow-up of 4.8 years from diagnosis. Patients with Wilms' tumor and malignant germ-cell tumors had the best outcomes (5-year OS 100% for both), whereas the worst prognosis was observed for sarcoma patients (5-year OS 72.92%). Premature infants had better outcome than full-term infants (5-year OS 92.8% vs. 72.58%, respectively).
Although rare, neonatal cancers can present with an aggressive clinical behavior, but they have a generally good outcome. Early diagnosis and management by expert multidisciplinary teams that integrate perinatal medicine experts with pediatric and surgical oncologists are critical. Centralized care with clear referral pathways that facilitate early initiation of specialized treatment should be prioritized.
在过去几十年中，儿科癌症多学科护理的进展使治愈率显著提高；然而，这些进展在所有年龄组中并不一致。癌症是围产期死亡的重要原因，但新生儿恶性肿瘤的全貌尚未明确界定。
作者回顾了1980年至2014年期间在波兰北部、中部和南部三个转诊中心接受治疗的37例先天性恶性肿瘤新生儿的临床特征和预后。采用Kaplan-Meier方法估计无事件生存率（EFS）和总生存率（OS），并使用长秩检验和Cox模型进行比较。
22例患者在产前被诊断。最常见的诊断是神经母细胞瘤（48.7%），其次是恶性生殖细胞肿瘤（16.2%）和肾母细胞瘤（8.1%）。神经母细胞瘤是足月儿中最常见的恶性肿瘤，而恶性骶尾部畸胎瘤是早产儿中最常见的恶性肿瘤。30例患者（81%）存活，自诊断起的中位随访时间为4.8年。肾母细胞瘤和恶性生殖细胞肿瘤患者的预后最好（两者的5年总生存率均为100%），而肉瘤患者的预后最差（5年总生存率为72.92%）。早产儿的预后比足月儿好（5年总生存率分别为92.8%和72.58%）。
尽管新生儿癌症罕见，但可表现出侵袭性临床行为，但其总体预后良好。由围产期医学专家与儿科和外科肿瘤学家组成的专家多学科团队进行早期诊断和管理至关重要。应优先考虑集中护理，并建立明确的转诊途径，以促进早期开始专科治疗。
