东莞地区1782例儿童活检病理分析

目的了解各种疾病在东莞地区儿童中的发病特点和规律。方法统计近5年来经病理检查证实1782例儿童活检病例,分析其性别、年龄、病理类型（先天性畸形、炎性反应性疾病、瘤样病变、良性肿瘤、恶性肿瘤）等发病特点。结果炎性反应性疾病占首位（44.61%）,瘤样病变占第2位（25.03%）,良性肿瘤占第3位（23.34%）,先天性畸形占第4位（5.22%）,恶性肿瘤占第5位（1.80%）。在炎性反应性疾病中以阑尾炎、慢性扁桃体炎及结核病多见;瘤样病变中以囊肿性病变及息肉性病变多见;良性肿瘤以脉管性肿瘤、毛母质瘤及骨软骨肿瘤多见;先天性畸形以耳前瘘管、肠憩室及肠重复多见;恶性肿瘤以软组织肉瘤、恶性外周神经肿瘤及霍奇金病多见。结论儿童疾病发生在年龄、性别及病理类型等方面区别于成人,有其自身特点。     

卵巢病变82例的诊断与治疗

目的 总结分析儿童卵巢病变的发病趋势、临床特点及诊治进展.方法 对本院1992年1月-2007年1月收治的82例卵巢病变息儿年龄、是否急诊、居住地、病理及手术方式等进行回顾性分析.结果 女性患儿82例.年龄1 d～14岁,平均年龄6.7岁.急诊入院31例(37.8%),平诊入院51例(62.2%);乡村患儿27例(32.9%),城镇患儿55例(67.1%);非肿瘤性病变45例(54.8%),良性肿瘤性病变31例(37.8%),恶性肿瘤性病变6例(7.4%).其中1992-1996年发病12例(14.6%),1997-2001年24例(29.5%),2002-2007年46例(55.9%).恶性肿瘤6例转内科化疗,性早熟2例保守观察,余74例均治愈.随访54例均无复发.结论 儿童卵巢病变可发生于任何年龄,以急腹症入院较多,城镇发病多于乡村,有逐年上升趋势,病理类型以良性为主,腹腔镜手术有明显优势.     

儿童肝脏局灶性结节性增生的病因机制及诊治进展

正肝脏局灶性结节性增生(focal nodular hyperplasia,FNH),是发生于正常肝组织背景上的单发或多发结节,由增生的肝实质组成的病变,是一种较少见的良性瘤样病变。FNH可见于任何年龄,在成人中比较多见,儿童中比较少见,约占儿童肝脏肿瘤的2%。在小儿肝脏良性肿瘤中,FNH的发病率低于肝脏血管瘤,居于第2位~([1])。目前认为FNH没有恶性转化倾向。众多研究表明,FNH越来越多见于恶性肿瘤史和造血干细胞移植史的病人。FNH     

肿瘤

530804 儿童肿瘤1123例统计分析冯淑文等哈尔滨医科大学学报19(3):47～49,19851123例,男599例,女524例。良性肿瘤616例(男285例,女331例)其中软组织肿瘤(主要为脉管瘤)373例,占60.6%,为第一位,先天残留组织中肿瘤74例,占125,居第二位;上皮瘤和神经系统肿瘤分别占11%和0.6%,居第三位。恶性肿瘤507例(男314例,女193例),其中白血病(270/507)占第一位,神经系统肿瘤(115/507)居第二位,淋巴系统肿瘤(39/507)居第三位。     

儿童及青少年原发性心脏肿瘤52例临床病理分析

目的 探讨儿童及青少年中罕见的原发性心脏肿瘤的组织学类型和病理特征,为临床诊断治疗提供形态学依据.方法 1976-2006年从中国医学科学院阜外心血管病医院近30年手术切除的562例原发性心脏肿瘤患者中,选择年龄不超过18周岁的病例.分析其临床病理所见,部分病例做了免疫组织化学和透射电镜检查.结果 52例中年龄平均12岁,最小6个月;男女各26例.良性肿瘤48例,占93%,其中黏液瘤最常见(31例),其它依次为纤维瘤(4例)、横纹肌瘤(3例)、海绵状淋巴管瘤(3例)、海绵状血管瘤(2例)、脂肪瘤(2例),还有颗粒细胞瘤、神经纤维瘤、平滑肌瘤各1例.恶性肿瘤4例,占7%,分别为血管肉瘤、纤维肉瘤、黏液纤维肉瘤及低分化肉瘤.3岁以内婴幼儿原发心脏肿瘤以横纹肌瘤(3例)和纤维瘤(3例)较多,还可见恶性的低分化肉瘤(1例).52例临床诊断与病理诊断符合或基本符合的占93%,误诊7%.结论 儿童心脏肿瘤类型多样,年龄较大者也以黏液瘤最多,而婴幼儿期以横纹肌瘤和纤维瘤多见.恶性肿瘤为分化较差的肉瘤,好发于右心,可见于婴幼儿.肿瘤临床诊断时应密切结合患儿年龄、瘤体生长部位及影像形态特点,才能减少误诊.     

4441例住院患儿肿瘤性疾病分布特征

目的分析4441例住院患儿肿瘤性疾病的分布特点。方法统计1993年1月~2004年12月我院信息科病案首页资料数据库按ICD-10标准登记18岁以下首次住院检出的肿瘤患儿,分析各肿瘤性疾病的顺位及构成比;患儿的年龄及性别特点;肿瘤性疾病在全年住院儿童中的检出率,比较各年度检出率的变化规律及发展趋势。结果12年内共检出各类肿瘤患儿4441例,男女之比1.4∶1,恶性、良性及动态未定肿瘤之比为0.24∶0.7∶0.06;良性软组织肿瘤是儿童最常见的良性肿瘤;恶性肿瘤中以淋巴、造血系统和有关组织肿瘤占首位,其次为起源于胚胎组织的神经母细胞瘤;不同瘤种患儿的年龄和性别有一定的特点,14岁以上的儿童患肿瘤明显减少。各年度肿瘤的检出率相对稳定,较接近平均水平(1.45%),近两年来患病绝对数和检出率有小幅增加。结论儿童患肿瘤性疾病有别于成人的自身特点,儿童以良性肿瘤居多,其中以血管瘤居首位;儿童恶性肿瘤以白血病为最多;儿童动态未定和未知肿瘤以颅内及椎管内肿瘤占首位;近年来14岁以内儿童肿瘤有增多趋势。     

��7��­������(��)

(上接本刊2006年第6期477页)4表皮样囊肿、皮样囊肿和畸胎瘤表皮样囊肿、皮样囊肿和畸胎瘤属先天性肿瘤。其中表皮样囊肿较多见,又称表皮样瘤、胆脂瘤、真性胆脂瘤或珍珠瘤,由残留原始外胚层细胞发展而来。占中枢神经系统肿瘤的1.39%。男性略多于女性,男女比例为1.2∶1。各年龄段均可发病,以20～50岁为最多见。皮样囊肿比表皮样囊肿更少见,约占颅内肿瘤的1%,可发生于任何年龄,20岁以下多见。畸胎瘤只占颅内肿瘤的0.5%,可发生于任何年龄,但小儿和青年人约占70%,男女比例为2.06∶1。4.1临床与病理表皮样囊肿常位于中线外侧。可位于脑内,如第四…     

儿童脊柱肿瘤

目的 探讨儿童脊柱肿瘤的临床特点、诊断、治疗方法及预后。方法回顾性分析我院1991年1月-2003年10月治疗的16岁以下脊柱肿瘤及瘤样病变44例，男27例，女17例；平均9．6岁。良性肿瘤（包括瘤样病变）38例，恶性肿瘤3例，转移瘤3例。疼痛39例，肿块9例，侧弯或斜颈8例，不同程度神经损害14例。18例初诊不确定或与最后诊断不符。发病至确诊时间平均8．5个月。手术治疗17例，化疗10例，放疗8例，手术与化疗1例，手术与放疗2例，放弃治疗3例，观察3例。结果围手术并发症4例。35例获平均5．5年随访。良性肿瘤及瘤样病变患儿手术或放疗或化疗后疗效较满意，所有患儿的局部疼痛消失，除1例外，所有患儿神经功能均有改善，3例病变复发再次手术。4例出现后弯畸形。6例恶性肿瘤和转移瘤患儿全部死亡。2例嗜酸性肉芽肿患儿症状消失，病变得到控制。结论对于儿童脊柱肿瘤，根据肿瘤的性质选择恰当的治疗方法，良性肿瘤与肿瘤样病变预后良好，恶性肿瘤及转移瘤治疗效果欠佳。     

儿童脊髓内肿瘤81例

目的总结儿童脊髓内肿瘤的病理特点、临床表现和手术治疗效果,探讨其特点和规律。方法81例儿童髓内肿瘤均经显微手术切除和病理证实,进行回顾性分析。结果儿童髓内肿瘤以星形细胞瘤24例(29.6%)、室管膜瘤13例(16.0%)和表皮样囊肿11例(13.6%)为多见,其运动系统损害是儿童髓内肿瘤最常见首发症状,其次为疼痛、感觉障碍、括约肌功能障碍等。放疗用于高度恶性肿瘤。椎板成形术可以有效减少脊柱畸形发生率。结论运动系统损害是儿童髓内肿瘤最常见首发症状,感觉障碍较成人发生率低。积极手术切除能获得满意疗效。     

婴幼儿和儿童心脏肿瘤的临床特点和超声诊断的应用价值

目的评价婴幼儿及儿童心脏肿瘤的临床特点、手术所见、病理学类型、以及治疗情况;评价超声心动图诊断婴幼儿及儿童心脏肿瘤的准确性。方法1983年12月至2003年12月在北京阜外心血管病医院手术和病理证实为心脏肿瘤的患者19例,男8例,女11例;年龄5个月~14岁,平均(7±5)岁。结果19例患者中,良性肿瘤17例(89.5%),其中黏液瘤10例(52.6%),横纹肌瘤4例(21.1%),纤维瘤2例(10.5%),脂肪瘤1例(5.3%);恶性肿瘤2例(10.5%),其中横纹肌肉瘤和转移性上皮肉瘤各1例。19例肿瘤中,11例位于左心(57·9%),7例位于右心(36.8%),1例位于双心室(5.3%)。术前超声10例诊断黏液瘤和1例怀疑横纹肌瘤与术后病理结果一致(11/19,57.9%),其余8例因超声缺乏经验,诊断为心脏占位病变性质待定或黏液瘤可能性大(8/19,42.1%)。结论婴幼儿和儿童心脏原发肿瘤多为黏液瘤,其次为横纹肌瘤,纤维瘤和脂肪瘤。黏液瘤多见于较大儿童,横纹肌瘤多见于婴幼儿,可呈多发性生长。当临床表现严重并难以控制时应选择手术。     

Oral tumors and tumor-like lesions in infants and children

The aim of this retrospective study was to survey the spectrum of oral tumors and tumor-like lesions treated in a pediatric surgical unit. The clinical features and treatment outcome are presented, and guidelines for management discussed. Long-term follow-up was carried out both by re-examination and by means of a questionnaire. A total of 95 patients were encountered over a 30-year period. The age at presentation ranged from 1 day to 16 years, and the male to female ratio was 0.7:1. The lesions were located predominantly on the lips (22%), tongue (21%), and cheek (19%). Patients were divided into five groups based on histological diagnosis. Benign lesions accounted for 83 (87%) of the cases. Of these, 41 (43%) were benign tumors, the most common of which were the hemangiomas (17 cases). Hamartomas accounted for a further 22 benign lesions (23%), among which 12 were lymphangiomas. Furthermore, we saw 14 cases (15%) of mucoceles, ranula and dysontogenetic cysts, and a further 6 cases (6%) were classed as miscellaneous lesions. Simple surgical resection was successful in treating most benign lesions, with occasional re-excision being necessary in lymphangiomas. The long-term effects of treatment include reduction of the red volume of the lips, scarring following resection of parotid hemangiomas, a forked tongue after wedged resection, and partial facial nerve palsy. The group of 12 (13%) malignant tumors consisted of 5 rhabdomyosarcomas, 2 fibrosarcomas, 2 carcinomas of the parotid, 1 osteosarcoma, and 2 metastases. A multimodal approach was used in patients with rhabdomyosarcomas, while fibrosarcomas and parotid carcinomas were normally treated by surgical excision. Six of 12 patients with malignant tumors were alive after a median follow-up of 20.5 years. Re-examination of the malignant tumor group revealed scarring, impaired growth and function of the maxilla associated with local irradiation, and an external salivary fistula. In conclusion, while most oral and maxillofacial tumors of children are benign, malignant tumors of soft tissue, salivary glands and bones must be taken into account. There are specific aspects related to certain developmental and biological characteristics that make a mainly conservative approach preferable in these children.     

Solid tumors in Turkish children: a multicenter study

Background

This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions.

Methods

The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out. Excluding epithelial tumors of the skin, leukemia, lymphoreticular system neoplasias, metastatic tumors, and hamartomas, 1362 solid tumors in 1358 patients were classified according to age, sex, localization, histopathology and clinical behavior.

Results

The male/female ratio was 0.9; 14.8% (201) of the patients belonged to 0-1 year age group, 20.7% (281) to 2–4 years, 25.9% (352) to 5–10 years, 22.2% (301) to 11–14 years, and 16.4% (223) to 15-18 years. Among all tumors, 708 (52.0%) were malignant, 645 (47.4%) benign tumors, 2 (0.1%) borderline tumors, and 2 (0.1%) unknown behavioral tumors. Malignant tumors were found in 50.2% (357) of female patients and in 54.0% (349) of male patients. A balanced distribution between benign and malignant entities among children under 18 years was observed. Comparison between the age groups revealed malignant cases outnumbered benign cases under 4 years of age while benign tumor numbers increased after 10 years of age. The most common entities in the malignant group were of sympathetic nervous system origin, while soft tissue tumors far outnumbered the others in the benign group.

Conclusions

We conclude that the cancer patterns of children in the Ankara region mostly resemble with those of the western population. This study provides useful information on the diagnosis of solid tumors in children and highlights variations in cancer incidence in different age groups.     

Congenital Malformations: A Review of 672 Autopsies in Ibadan, Nigeria

The postmortem findings in 672 Nigerian children with lethal congenital malformations are reviewed. Eighty-six percent of the patients were less than 1 year of age and the male-to-female sex ratio was 1.3:1. The alimentary tract was the most common anatomic site involved (30%), intestinal and biliary atresia being the two lesions most frequently identified in this location. Twenty-six percent of the children had cardiovascular malformations, of which ventricular septal defect, patent ductus arteriosus, and tetralogy of Fallot were most important. There was an accentuated male predominance among cases with urogenital malformations due to the high frequency of posterior urethral valve. Hydrocephalus, spina bifida, and anencephaly were the most common lesions observed in the craniospinal axis. One-fifth of the children had complex malformations of multiple organ systems, 20% of which were associated with chromosomal anomalies, rubella, and other malformation syndromes.     

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。     

Childhood mediastinal masses in infants and children

We reviewed all cases of primary pediatric mediastinal masses diagnosed and treated over a 24-year period. In this study, out of 187 primary mediastinal mass cases diagnosed between 1980 and 2004 in Istanbul University Istanbul Faculty of Medicine, Cardiovascular Surgery Department, 37 pediatric primary mediastinal mass cases were retrospectively evaluated according to age, sex, symptoms, diagnostic procedure, anatomical location, surgical treatment, histopathological evaluation and postoperative adjuvant therapy. The patients ranged in age from 2 months to 15 years at the time of diagnosis, with a mean age of 8 years. There were 24 benign (64.8%) and 13 malignant (35.2%) tumors. The cases were lymphoma (27%), neurogenic tumors (21.6%), cystic lesions (18.9%), germ cell tumors (13.5%), thymic lesions (10.8%) and cardiac tumors (8.1%). Complete and partial resections of the tumor were the surgical procedures performed in 24 patients (64.8%) and 3 patients (8.1%), respectively. The three patients with a malignant tumor, in whom the entire mass could not be removed, received chemotherapy and radiation after surgery. In 10 patients with lymphoma, surgery was not a part of treatment and they received medical and radiation therapy after the establishment of the definitive diagnosis. All patients survived and were discharged from the hospital. Except for the cases with lyphoma, all patients are now free of recurrent disease. Compared to adults, children had more lymphomas and neurogenic tumors. Primary pediatric mediastinal malignancies are relatively common in infants and children. Lymphoma, neurogenic tumors and cystic lesions predominated. These differences between the age groups should also be considered when dealing with a mediastinal mass.     

儿童及青少年卵巢肿瘤187例

目的 探讨儿童及青少年卵巢肿瘤的发病特点、病理类型与发病年龄的关系,并评价其诊治手段及预后因素.方法 选取1980年1月-2005年12月在中山大学肿瘤防治中心治疗并确诊的儿童及青少年卵巢肿瘤患儿187例,并对其临床及随访资料进行回顾性分析.采用SPSS 13.0统计软件,定性变量比较采用χ2检验,生存率计算用寿命表法.采用Kaplan-Meier法进行生存分析.结果 187例卵巢肿瘤中良性56例,恶性119例,交界性12例.不同病理类型的卵巢肿瘤与患儿发病年龄有一定关系(χ2=12.396,P=0.015).187例卵巢肿瘤中,56例良性肿瘤5 a总生存率为98%,119例恶性肿瘤5 a总生存率为76%,12例交界性肿瘤5 a生存率为83%.单因素生存分析显示,影响总生存时间的危险因素为肿瘤分期、是否行规范化疗和化疗方案.结论 超声及CT是卵巢肿瘤诊断的主要方法.不同病理类型的儿童卵巢肿瘤与发病年龄有一定关系.肿瘤分期、术后辅助治疗方式均影响患儿总生存时间.     

Tumores testiculares y paratesticulares en la infancia y adolescencia

IntroductionTesticular and paratesticular tumors represent 1-2% of the solid tumors in children. We present a retrospective series of 15 cases in patients less than 18 years of age.ResultsThe mean age of the patients was 9.7 yrs, 6 of them prepubertal (mean age: 2.08 ± 1 yrs) and 9 pubertal (mean age: 15.1 ± 1.3 yrs). The most common clinical form of presentation was a painless testicular mass. The α-fetoprotein levels were high in 5 patients (yolk-sac tumors and embryonal carcinomas).The pathological study showed 11 primary testicular tumors and 4 paratesticular tumors (rhabdomyosarcomas), with 60% being germinal tumors and the rest non-germinal. Around 60% were malignant tumors (2 from the yolk-sac tumors, 2 embryonal carcinomas, one seminoma and 4 rhabdomyosarcomas). Among the benign tumors, the most common was the mature cystic teratoma. Surgery was the initial treatment in all of the cases (radical orchiectomy in 13 tumors and enucleation in 2 teratomas, with retroperitoneal lymphadenectomy in 4 cases). In 11 patients the tumor was in stage I, while 4 cases (2 embryonal carcinomas and 2 rhabdomyosarcomas) were in stage IV with pulmonary metastasis. Chemotherapy whether or not combined with radiotherapy was applied in 7 patients (4 rhabdomyosarcomas, 2 embryonal carcinomas and one seminoma).ConclusionsTesticular and paratesticular tumors in prepubertal children show epidemiological, histological, therapeutical and evolutional characteristics well differentiated from postpubertal or adult subjects.     

A Clinico-Pathologic Study of 142 Orofacial Tumors in Children and Adolescents in Southern Iran

Objective

The prevalence, patients’ age and sex and the site of the lesions are important factors for diagnosis and they may be different in various populations. The aim of this study was to determine the type and distribution of orofacial tumors among children and adolescents in an Iranian population

Methods

In this retrospective, case series study, data about the type, age, sex and site of 142 tumors in patients ≤18 years afflicted with orofacial neoplasms referred 2005-2009 to two referral centers in Shiraz, Southern Iran, were collected and analyzed.

Findings

There were 142 (2.8%) tumors among the subjects. The most common types of benign and malignant tumors were odontoma and lymphoma in children and pleomorphic adenoma and rhabdomyosarcoma in adolescents. Parotid and posterior parts of the mandible were the most common sites of soft tissue and intrabony tumors. In the oral cavity, the palate was the most common affected site. The tumors were found in boys with higher frequency (Male:Female ratio was 1.4:1).

Conclusion

The observed differences in tumor type and distribution in comparison with previous studies may be attributed to genetic and geographic variations in the populations; however the design and methods of the studies are different, too.     

Orbital space-occupying lesions in Indian children

PURPOSE: To present a review of all patients younger than 16 years who presented to us with orbital space-occupying lesions. METHODS: Retrospective case study. RESULTS: We analyzed the records of 119 children younger than 16 years of age who had presented to us with proptosis during the 10-year study period. Myogenic tumors were the most common lesion seen (24%), followed by metastatic and secondary orbital tumors (17%), vasculogenic lesions (15%), cystic lesions (10%), inflammatory lesions simulating tumors (7.5%), optic nerve and meningeal tumors (7.5%), peripheral nerve tumors (5%), lacrimal fossa lesions (4%), lymphoid tumors and leukemias (2.5%), primitive neuroectodermal tumors (3%), and others (2.5%). CONCLUSION: We analyzed the profile of orbital lesions that underwent surgical procedures and were proven histopathologically. We found 63 malignancies among the 119 cases, for a malignancy rate of 53%. Orbital rhabdomyosarcoma and retinoblastoma with orbital spread were the most common causes of proptosis among the children.