自噬调控在神经母细胞瘤中的作用研究进展

神经母细胞瘤是儿童和婴儿时期常见的实体肿瘤之一,早期即可发生转移,恶性度极高。本病的治疗以手术联合化疗为主,但预后不理想。因此寻找新的治疗靶点是当今的研究热点之一。近年来研究发现,细胞自噬调控异常与肿瘤的炎症反应、能量代谢、细胞程序性死亡及对肿瘤治疗的耐药性等方面有密切联系。因此,本文就细胞自噬调控与神经母细胞瘤之间的关系作一综述,旨在为神经母细胞瘤防治工作提供新的思路。     

婴儿实体肿瘤的发病特点与特殊处理

目的 探讨婴儿实体肿瘤的发病特点、治疗原则及预后。方法 回顾性研究1995-2000年间68例婴儿实体肿瘤的病理类型、治疗方法和生存率及死亡原因。结果 恶性肿瘤40例（58．8％），良性肿瘤多为畸胎瘤。恶性肿瘤手术切除率83．3％。29例接受化疗，其中79．3％予30％～70％的化疗减量。婴儿恶性肿瘤3年生存率66．7％，5年生存率45．4％。结论 婴儿肿瘤的主要治疗手段为手术切除辅以术后化疗，适当的化疗减量必要、安全、有效。     

儿童恶性实体肿瘤需要多学科合作的规范化治疗

儿童恶性实体肿瘤至今仍是导致儿童死亡的重要原因之一,发病率呈上升趋势,随着交通科技的发展和新农村合作医疗体系的推广,越来越多的患儿获得了治疗的机会。各大儿童医疗中心近年来收治儿童肿瘤病例明显增加。在中华小儿外科学会肿瘤学组和中国抗癌协会儿童抗癌分会等组织的努力下,全国范围的多中心合作、多学科合作及规范化诊治已具雏形。儿童肿瘤的临床科研水平也较前有了显著提高。现对我国近10年来儿童肿瘤的临床诊疗进展作一评述。     

儿童恶性实体瘤诊断与治疗现状

国际儿童实体瘤多中心协作研究、多学科协同工作模式对儿童实体瘤预后进步有重要影响,以青少年/儿童恶性生殖细胞瘤、长春花碱对儿童间变大细胞淋巴瘤预后影响、骨肉瘤随机分组研究维持治疗的有效性多中心临床研究结果为例进行探讨。对放疗、造血干细胞移植、靶向治疗等在儿童实体瘤儿童肿瘤综合治疗中地位作用进行介绍。同时讨论了我国儿童实体瘤诊治现状和加以改善的期望。     

严重急性呼吸综合征冠状病毒2流行期间儿童实体肿瘤诊治中的防控策略

自2019年12月湖北出现新型冠状病毒感染(corona virus disease 2019,COVID-19)患者以来,该病毒现已逐步扩散至全国各地及境外多个国家。国际病毒分类委员会将致病病毒命名为严重急性呼吸综合征冠状病毒2(severe acute respiratory syndrome coronavirus 2,SARS-CoV-2),人群普遍易感,包括儿童和孕产妇。恶性实体肿瘤患儿因肿瘤本身及化疗、放疗、免疫治疗等综合治疗手段的影响,免疫力较正常儿童低,可能更容易发生感染并进展为重症病例,而保障恶性实体肿瘤综合治疗计划实施的延续性和规范性是提高疗效、获得良好预后的关键之一。疫情期间,如何在做好疫情防控工作的同时保障肿瘤诊疗工作有序进行,是摆在每一个病患和医务人员面前的难题。本文结合COVID-19的传播特点以及有关部门防控COVID-19的要求,针对儿童实体肿瘤诊疗工作中的主要环节,提出疫情期间应考虑实施的措施和策略,建议制定科学的实体肿瘤门诊和病房管理制度,保障急诊手术,合理安排限期手术,适当延期择期手术,保障化疗规律进行,在保护患儿免受SARS-CoV-2感染的同时,确保儿童实体肿瘤综合诊疗工作的连续性。     

神经母细胞瘤分子遗传学研究进展

神经母细胞瘤（neuroblastoma,NB）是儿童最常见的实体肿瘤,约占儿童肿瘤的8%～10%,是学龄前儿童癌肿死亡的主要原因.其重要特性是表型和遗传异质性.已知的预后因素有年龄、发病部位、分期、肿瘤分化程度、DNA含量和不同遗传学改变等.这些预后变量的结合可用于风险评估和治疗分层.近年来新的研究方法和研究成果为该病的风险评估和靶向治疗开启了新的机遇.本文就已发现的一些重要的分子遗传学畸变作一简要综述.     

神经母细胞瘤治疗进展

<正>神经母细胞瘤(neuroblastoma,NB)来源于肾上腺髓质或交感神经节,是儿童最常见的颅外实体肿瘤,年发病率约0.3/10万~5.5/10万,占儿童肿瘤的7%~8%。NB占因肿瘤致死的儿童15%~([1])。我国流行病学资料显示,NB的发病率呈上升趋势,每年新诊约3000例,仅次于白血病及中枢神经系统肿瘤疾病。由于其恶性程度高、易发生转移等特点,约70%~80%的患者确诊时已是晚期(Ⅲ期或Ⅳ期),预后差。近20年来,国际上对儿童NB的治疗已形成较为规范的诊疗指南,国内肿瘤学组也初步达成专家共识,低-中危组患儿能获得良好预后,长期无     

儿童恶性肿瘤275例临床病理分析

目的：了解该院近10年来儿童恶性肿瘤(除外白血病)的概况。方法：对该院1991～2000年10年间儿童实体肿瘤1 236例进行回顾性分析。结果：儿童实体恶性肿瘤共计275例,占全部实体肿瘤的22.25%,其中男性167例,女性108例,男女之比 1.55∶1;年龄最小者为2个月,最大的14岁;占前3位的分别是恶性淋巴瘤58例(21.09%),软组织肉瘤36例(13.09%),肾母细胞瘤32例(11.65%),按发病年龄分组看,恶性肿瘤从婴儿组,幼儿组,学龄前组到学龄组患者有逐渐增多的趋势,但某些恶性肿瘤如：恶性淋巴瘤、肾母细胞瘤、神经母细胞瘤等也有相对集中的发病年龄。结论：儿童恶性肿瘤的发病在年龄及性别等方面有其一定的规律性。     

microRNA在神经母细胞瘤的研究进展

神经母细胞瘤是儿童最常见的颅外恶性实体肿瘤,肿瘤转移及复发是其死亡的重要原因.神经母细胞瘤的发生与胚胎发育异常密切相关,但其确切的发病机制、分化成熟及转移的机制并未完全阐明.微小RNA在机体胚胎发育过程和肿瘤的发生中起到重要作用,并与肿瘤发展及预后密切相关.该文就微小RNA在神经母细胞瘤的研究进展作一综述.     

嵌合抗原受体T淋巴细胞治疗神经母细胞瘤的研究进展

嵌合抗原受体T淋巴细胞(chimeric antigen receptor T lymphocyte,CAR-T)免疫治疗是近年来开始出现的一种新型肿瘤免疫治疗方法,目前已经在白血病、淋巴瘤等晚期癌症患者中应用,并取得一定疗效.神经母细胞瘤是儿童最常见的颅外恶性实体肿瘤之一,有超过一半的患者在确诊时已经发生了转移.目前高危患儿的预后较差,临床需要寻找新的治疗方法.该文总结了CAR-T的基本结构及优化发展,讨论了其在神经母细胞瘤中的治疗原理及风险,并展望了CAR-T在实体肿瘤中的治疗前景.     

Magnetic resonance imaging features of desmoplastic cerebral ganglioglioma of infancy: report of 1 case]

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of infancy, characterized by solid and cystic component, voluminous size and supratentorial location. These tumors are diagnosed usually below the age of 2 years. We report 1 case of desmoplastic ganglioglioma in 13-year-old male. Computed tomography and magnetic resonance imaging diagnosed supratentorial mixed cystic and solid tumor, which presented as a large cystic component with intense contrast enhancement of a mural nodule. The tumor was surgically removed, and histology revealed desmoplastic ganglioglioma. The patient had a good follow up. This observation emphasizes the possibility of desmoplastic ganglioglioma in older infants. It mustn't be considered as a specific entity of very young age infant and must be recognized in older infant because it may be misdiagnosed as malignant glioma. Despite the pseudo malignant appearance, these tumors have a good prognosis after surgery and when excision is complete they don't led to recurrences.     

A Single Center Experience in 266 Patients of Infantile Malignancies

Introduction: The diagnosis and management of cancer in infantile age group is a significant challenge to pediatric oncologists. Malignancies occurring in infants often have different clinical and biological behavior in comparison to older children. This study was performed with an aim to find out the profile of infantile cancers at a tertiary care cancer center in South India. Methods: The present study was a retrospective analysis of infants presenting with malignancy between 2003 and 2012 to our center in South India. Result: A total of 4588 pediatric patients were registered in the Department of Pediatric Oncology at our institute between 2003 and 2012. Among those, 266 (5.79%) of the patients were infants (0–1 years). There were 65.75% males and 34.25% females. Solid tumors were the most common malignancy in this age group (72.56%). Leukemias were observed in 67 (25.19%) infants. ALL was the most common hematological malignancy (17.29%) followed by AML (5.64%). Common solid tumors in descending orders were neuroblastoma, soft tissue sarcoma, renal tumors, germ cell tumors, retinoblastoma and hepatoblastoma. Thirteen (4.89%) neonates were seen in our study. The most common malignancy in neonates was neuroblastoma. Conclusion: The distribution of malignancy in infants is quite different from that which is found in older children. Although neuroblastoma is the most common infantile tumor in western countries, in our study leukemia is the most common infantile malignancy. Embryonal tumors such as neuroblastoma, Wilms tumor, retinoblastoma, and hepatoblastoma were more prevalent in infants. Solid tumors were the most common malignancy in infants which is followed by leukemia.     

单中心2009至2011年住院患儿中实体肿瘤状况分析

目的 分析儿童实体肿瘤的原发部位和病理类型,为儿童实体肿瘤的诊治提供帮助。方法 回顾性收集2009年1月至2011年12月经手术/病理活检确诊的实体肿瘤患儿,采集肿瘤原发部位、病理类型和组织起源等资料,分为0～1、~4、～7、～10和～14岁组进行分析。结果 983例实体肿瘤患儿进入分析,其中良性肿瘤721例（73.3%）,恶性肿瘤262例;男548例（55.7%）,女435例;发病年龄8 d至14岁,中位年龄4岁。①721例良性肿瘤中,以脉管瘤（233例,32.3％）、钙化上皮瘤（161例,22.3％）、软骨瘤（68例,9.4％）、纤维瘤（67例,9.3％）和成熟畸胎瘤（66例,9.2％）多见。262例恶性肿瘤中,以神经母细胞瘤（44例,16.8％）、恶性淋巴瘤（34例,13.0％)和肾母细胞瘤（33例,12.6％)多见。②良性肿瘤好发于皮肤及软组织（324例,44.9％）、头面颈部（220例,30.5％）和骨骼（86例,11.9％）。恶性肿瘤好发于腹腔（96例,36.6%）、颅内（51例,19.5％）和头面颈部（33例,12.6％）。③恶性肿瘤以胚胎源性肿瘤最多见（171例,65.3％）,其次为含类似组织成分的肿瘤（57例,21.8％）和淋巴源性肿瘤（34例,13.0％）。④良性肿瘤中,脉管瘤、钙化上皮瘤和纤维瘤主要分布于~4岁组。恶性肿瘤中,神经母细胞瘤和肾母细胞瘤在0～1岁和～4岁组累计构成比分别为84.1％和81.9％,恶性淋巴瘤在4岁以上组累计构成比为71.6％。⑤巨大恶性肿瘤29例（11.1%）,其中24/29例（82.8%）年龄<4岁,腹腔为最常见原发部位（26例,89.7%）。结论 良性肿瘤是儿童实体肿瘤的主要构成。儿童恶性肿瘤好发于腹腔,组织来源以胚胎源性肿瘤为主。神经母细胞瘤及肾母细胞瘤可能是<4岁儿童腹腔巨大恶性肿瘤的主要病理类型。     

小儿实体肿瘤治疗的现状

小儿实体肿瘤的治疗是提高恶性实体瘤生存率的关键之一。肿瘤的外科处理除挽救生命外,主要考虑到生活质量。化疗是选择有效合理联合应用抗癌药,且副作用小,重点之一是化疗药物耐药性的研究;在儿童中尽量少用放疗,如必需应用,也需注意防护与适当剂量,现介入性治疗和术中放疗已逐渐开展。治疗实体瘤的新措施还包括生物反馈修饰剂、诱导肿瘤分化、靶向药物和单克隆抗体等。     

The role of neoadjuvant chemotherapy in children with malignant solid tumors

Pediatric surgeons play a critical role in diagnosing, staging, and treating malignant solid tumors in children. Over the years, the surgical management of the primary tumor site has evolved from an aggressive en-bloc resection at diagnosis to a more tailored surgical approach, often affecting definitive local control after the delivery of neoadjuvant therapy, as currently directed by many solid tumor protocols. In fact, inappropriate upfront resection can lead to unnecessary short- and long-term morbidity, an incomplete resection, and may be associated with a delay in the initiation of the systemic chemotherapy that is critical to the treatment of gross or occult metastatic disease. Therefore, it is important for the pediatric surgeon, as a member of the multidisciplinary team involved in the care of these children, to understand the indications for and implications of neoadjuvant therapy in the treatment of pediatric solid tumors. Here we review the current management of childhood solid tumors focusing on the role of neoadjuvant therapy.     

小儿实体肿瘤310例分析

目的 总结各种类型小儿实体肿瘤的诊治以及各年龄段的大致分布情况。方法 选择1996年1月-2006年1月,我院收治的310例小儿实体肿瘤患儿病例资料,对其年龄分布、诊断及治疗进行回顾性分析。结果 310例患儿中,良性肿瘤147例,占总病例数的47．4％;恶性肿瘤163例,占总病例数的52．6％。男：女=1．25：1。年龄在1岁以内126例,占总病例数的40．7％;1。3岁93例,占30％;7-14岁44例,占14.2％。根据肿瘤类型和病人的个体差异分别采取手术、化疗、放疗以及支持疗法等。治疗不同,疗效各异。结论 小儿恶性肿瘤除需采取积极的手术治疗外,尚需要化疗、放疗以及营养支持治疗等多种方法的紧密配合。     

婴儿腹腔实体肿瘤临床病理与预后分析

目的探讨婴儿腹腔实体肿瘤的临床病理与预后的关系。方法回顾上海儿童医学中心1998年4月-2007年2月及上海新华医院2001年1月-2007年2月住院的52例1d～1岁腹部肿瘤患儿临床资料。患儿病史资料通过病史采集方式获得。病理类型通过复查病理切片和病理报告核对。随访采用电话和信访方式及患儿来院复诊记录。随访时间5个月～8a。结果52例中年龄1d～1个月7例,≥1个月～1岁45例。其中畸胎瘤23例(44.23%),神经母细胞瘤9例(17.31%),肾母细胞瘤6例(11.54%),肝母细胞瘤5例(9.62%),肝血管内皮瘤、先天性中胚层肾瘤各3例(5.77%),胰头梭形细胞血管内皮瘤、肝错构瘤、后腹膜小细胞恶性肿瘤各1例(1.92%)。良性肿瘤:恶性肿瘤=1:1,良性肿瘤中男:女=1:1,恶性肿瘤中男:女=2.33:1.0。患儿均手术切除肿瘤,恶性肿瘤辅以化疗。良性肿瘤均无复发;恶性肿瘤中复发或转移2例,其中1例死亡。失访4例。结论婴儿腹腔实体肿瘤以畸胎瘤和神经母细胞瘤居多。良性肿瘤数量与恶性肿瘤相当。良性发病无性别差异,手术切除后预后良好。恶性肿瘤中,男性比例显著高于女性,完整手术切除配合术后化疗,复发少,病死率低。早期诊断和治疗对其预后至关重要。     

Challenge of pediatric oncology in Africa

The care of children with malignant solid tumors in sub-Saharan Africa is compromised by resource deficiencies that range from inadequate healthcare budgets and a paucity of appropriately trained personnel, to scarce laboratory facilities and inconsistent drug supplies. Patients face difficulties accessing healthcare, affording investigational and treatment protocols, and attending follow-up. Children routinely present with advanced local and metastatic disease and many children cannot be offered any effective treatment. Additionally, multiple comorbidities, including malaria, tuberculosis, and HIV when added to acute on chronic malnutrition, compound treatment-related toxicities. Survival rates are poor. Pediatric surgical oncology is not yet regarded as a health care priority by governments struggling to achieve their millennium goals. The patterns of childhood solid malignant tumors in Africa are discussed, and the difficulties encountered in their management are highlighted. Three pediatric surgeons from different regions of Africa reflect on their experiences and review the available literature. The overall incidence of pediatric solid malignant tumor is difficult to estimate in Africa because of lack of vital hospital statistics and national cancer registries in most of countries. The reported incidences vary between 5% and 15.5% of all malignant tumors. Throughout the continent, patterns of malignant disease vary with an obvious increase in the prevalence of Burkitt lymphoma (BL) and Kaposi sarcoma in response-increased prevalence of HIV disease. In northern Africa, the most common malignant tumor is leukemia, followed by brain tumors and nephroblastoma or neuroblastoma. In sub-Saharan countries, BL is the commonest tumor followed by nephroblastoma, non-Hodgkin lymphoma, and rhabdomyosarcoma. The overall 5-years survival varied between 5% (in C?te d'Ivoire before 2001) to 34% in Egypt and up to 70% in South Africa. In many reports, the survival rate of patients is not mentioned but is clearly very low in many sub-Saharan Africa countries (Sudan, Nigeria). Late presentation was observed for many tumors like nephroblastoma in Nigeria, 72% were stages III and IV or BL stages III and IV were observed in 40% and 30%, respectively. Africa bears a great burden of childhood cancer. Cancer is now curable in developed countries as survival rates approach 80%, but in Africa, >80% of children still die without access to adequate treatment. Sharpening the needlepoint of surgical expertise will, of itself, not compensate for the major infrastructural deficiencies, but must proceed in tandem with resource development and allow heath planners to realize that pediatric surgical oncology is a cost-effective service that can uplift regional services.     

Pediatric tumors of the eye and orbit

Most ocular and orbital tumors of childhood are distinct from tumors that occur in adults. Many are congenital with early presentations. Most pediatric orbital tumors are benign; developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumor. The most common orbital malignancy is rhabdomyosarcoma. The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children. The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults. Pediatricians play a vital role in diagnosis of pediatric ocular tumors. They are the first to recognize ocular problems that may not be apparent to parents. It is therefore important to recognize the signs and symptoms of ocular tumors of childhood so that prompt ophthalmologic evaluation and treatment may be undertaken. Whereas the malignant tumors may be life-threatening, both malignant and benign tumors may be vision-threatening.     

Benign liver and biliary tract masses in infants and toddlers

There is a remarkable diversity of conditions encompassed by benign liver masses in infants and toddlers. The most common benign hepatic tumor in this age group is infantile hepatic hemangioendothelioma. Other commonly seen benign tumors are mesenchymal hamartoma and focal nodular hyperplasia. Hepatic adenoma is almost exclusively a disease of older children; primary hepatic teratoma is exceedingly rare. There are several distinguishing characteristics of these benign tumors on radiographic evaluation; however, imaging techniques such as ultrasound scan, computed tomography, and angiography are not always reliable in differentiating benign from malignant tumors. The differential diagnosis of benign hepatic tumors includes nonneoplastic cystic masses including biliary and simple hepatic cysts, hematoma, parasitic cysts, and pyogenic and amebic liver abscess. Choledochal cyst presents with a classic triad of abdominal pain, cholestatic jaundice, and a palpable abdominal mass. They are classified anatomically into 5 subtypes with the most popular types being type I and type IV. Treatment is with complete cyst excision with hepaticojejunostomy reconstruction.