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初现端倪的抗争——新生儿癌症:发病率低但预后良好,治疗颇具挑战

The Fight Just Born-Neonatal Cancer: Rare Occurrence with a Favorable Outcome but Challenging Management.

作者信息

De Ioris Maria Antonietta, Fabozzi Francesco, Lodi Mariachiara, Vitali Giulia, De Pasquale Maria Debora, Del Baldo Giada, Abbas Rachid, Agolini Emanuele, Crocoli Alessandro, Iacusso Chiara, Milano Giuseppe Maria, Serra Annalisa, Mastronuzzi Angela

机构信息

Department of Hematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Department of Pediatrics, University of Tor Vergata, 00165 Rome, Italy.

出版信息

Cancers (Basel). 2022 Apr 29;14(9):2244. doi: 10.3390/cancers14092244.

Abstract

The occurrence of cancer in newborns within the first 28 days of life is uncommon, with different clinical presentation from other age groups. Prenatal diagnosis is reported in about half of patients, while a genetic predisposition condition is supposed. The management of a newborn with cancer can be challenging and needs to be tailored according to the histology and the primary tumor site; surgery represents the main strategy, while chemotherapy should be considered with caution because of the higher toxicity and mortality due to different pharmacokinetics in neonates compared to older children. We describe the first Italian series over a 15-year period of patients affected by both benign and malignant neoplastic diseases diagnosed within the first 28 days of life; 74 newborns were diagnosed with neonatal tumors, representing 1.5% of the cancer population in the same period, and a prevalence of germ cell tumors (55%) and neuroblastoma (16%) was observed. Surgery was performed on 80% of patients, while chemotherapy was necessary for about 20% of patients. The 5-year overall survival (OS) exceeded 90%; treatment-related deaths are a major concern, representing 80% of overall deaths. A genetic/syndromic condition was detected in 16% of the population; additionally, a cancer predisposition syndrome (CPS) was identified in about 10% of patients. According to our experience, all newborns affected by cancer should warrant genetic counselling and a screening test for CPS.

摘要

新生儿在出生后28天内患癌症的情况并不常见,其临床表现与其他年龄组不同。约半数患者有产前诊断,同时推测存在遗传易感性疾病。癌症新生儿的治疗具有挑战性,需要根据组织学和原发肿瘤部位进行个体化治疗;手术是主要治疗策略,而化疗应谨慎考虑,因为与大龄儿童相比,新生儿的药代动力学不同,化疗毒性和死亡率更高。我们描述了意大利15年间首组出生后28天内诊断为良性和恶性肿瘤疾病的患者;74例新生儿被诊断为新生儿肿瘤,占同期癌症患者总数的1.5%,观察到生殖细胞肿瘤患病率为55%,神经母细胞瘤患病率为16%。80%的患者接受了手术治疗,约20%的患者需要化疗。5年总生存率超过90%;与治疗相关的死亡是主要关注点,占总死亡人数的80%。16%的患者检测到遗传/综合征性疾病;此外,约10%的患者被确定患有癌症易感综合征(CPS)。根据我们的经验,所有患癌症的新生儿都应接受遗传咨询和CPS筛查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c865/9103742/f52a49ea8bbd/cancers-14-02244-g001.jpg

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