Ultrasound diagnosis of multiple cystic encephalomalacia

A retrospective examination of brain tumors in infants less than a year of age was undertaken by reviewing their charts and CT scans. In contradistinction to brain tumors found in older children, most tumors were supratentorial in location. The most common histologic types included: astrocytoma, ganglioglioma and primative neuroectodermal tumors. Apart from their larger size at the time of presentation, these tumors were radiologically and pathologically similar to analogous tumors found in adults.     

Childhood tumors of the brain: demographic pattern over a ten-year period in the Kashmir Valley

Brain tumors in children represent the second most frequent tumors in this age group after hematologic malignancies. We highlight the demographic pattern after retrospective analysis of brain tumors in children from geographically and ethnically distinct Kashmir Valley managed in our center between 2000 and 2009. We had a total of 248 pediatric patients with brain tumors. The parameters analyzed were age, gender, location of tumors and histopathological subtypes as well as WHO grade of tumor. We also did a comparison between the frequencies of common varieties of tumor in the first and second 5-year periods. We found that 111 tumors (44.75%) were supratentorial, and 137 (55.25%) were infratentorial. The male-to-female ratio was 1.4:1. The proportions of low-grade and high-grade tumors were 60 and 40%, respectively. The most common tumor in our series was astrocytoma. The most common tumors in the supratentorial and infratentorial compartments were craniopharyngioma and medulloblastoma, respectively. Our experience reflects a different demographic profile of pediatric brain tumors as compared with other regions of the world.     

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。     

儿童胰腺肿瘤8例报告

目的 介绍儿童胰腺肿瘤的常见类型,提高对这些疾病的诊断和治疗水平.方法 报告 8 例儿童胰腺肿瘤,男 3 例,女 5 例,年龄2岁至14岁8个月.7 例患儿行手术治疗,其中 1 例良性畸胎瘤;低度恶性者 3 例(囊实瘤 2 例,胰岛细胞瘤 1 例);高度恶性者 3 例(胰母细胞瘤 2 例,胰头癌1 例);未手术 1 例.结果 7 例手术者中良性和低度恶性病例术后随访至今未见复发及不适;3 例高度恶性病例患儿术后均放弃进一步治疗;1 例未手术者6个月后死亡.结论 儿童胰腺肿瘤在临床上相对少见,不同病理类型临床表现不同,治疗方法 各异,预后也相差很大.     

Rare tumors in childhood: Pathological aspects. Experience of the Kiel pediatric tumor registry

A wide variety of rare tumors can occur in childhood and adolescence. Tumors can be rare in any age group; common tumors of adults can appear in childhood and adolescence, and many rare tumors and tumor-like lesions show a predilection for younger age groups. Comparatively frequent tumors can present with rare histologic features or can occur in rare atypical locations, and, finally, seemingly common but, in fact, rare tumors have to be distinguished from truly common but rarely recognized tumors. Thus, the spectrum of rare tumors is broad, reflecting the fluent reality of life. © 1993 Wiley-Liss, Inc.     

Problematic, clinic and treatment of benign bone tumors in childhood and adolescence (author's transl)

Bone tumors in children show a large variety in Kind. Often there is no clear distinction between benign and malignant tumors. So in between according to Zollinger there are semimalignant tumors with potential malignity. Adequate excision of these tumors or resection enbloc inhibit the tumor recurrence and give a good prognosis to semimalignant tumors. 31 children with benign bone tumors were operated between 1972 and 1979. They have been followed up medically and radiologically from 1 to 7 years. Up to now we could neither find a recurrence nor a sign of malignant degeneration.     

Adult type granulosa cell tumor causing precocious pseudopuberty in a 6 year-old girl

Malignant ovarian tumors are responsible for 2-3% of all cases of precocious pseudopuberty (PP) in girls. The most common forms of ovarian tumors presenting as PP are the granulosa cell tumors (GCT). The clinical and pathological features of granulosa cell tumors that occur frequently in young girls, so-called juvenile granulosa cell tumors (JGCT), differ histologically from those occurring in adults. As a cause of PP in young girls, adult type granulosa cell tumors (AGCT) are extremely rare. We report a 6 year-old girl presenting with early breast development and vaginal bleeding due to a well encapsulated ovarian tumor. Microscopic features of the resected tumor were characteristic of AGCT.     

三岁以下低龄儿童原发性脑肿瘤临床回顾与分析

目的 回顾性分析5年间我院3岁以下低龄儿童原发性脑肿瘤的临床、病理特点以及预后情况.方法 自2006年1月至2010年12月间,我院共收治87例3岁以下原发性脑肿瘤患儿,通过手术治疗的同时获得其组织病理学报告,根据WHO2007年新分类,分析该年龄组患儿的临床症状、组织学类型、病理分级、治疗方案的特点以及影响预后的相关因素.结果 该年龄组患儿以男性居多,脑肿瘤幕上多于幕下,并以中线部位多见.神经上皮肿瘤为最多见病理类型,按发病率排序的肿瘤类型依次为:低级别星形细胞瘤(20.7％)、髓母细胞瘤(13.8％)、室管膜肿瘤(12.6％)以及脉络丛乳头状瘤(12.6％).患儿的临床表现以头围增大(48.3％)、呕吐(36.8％)、肌力下降(19.5％)以及抽搐(16.1％)为主.预后与肿瘤位置、肿瘤病理、年龄以及手术切除程度有关.结论 3岁以下年龄组患儿的预后较大龄儿童差,手术治疗仍为低龄患儿的首选治疗方案,围手术期处理以及术后辅助治疗的加强为今后工作研究的重点.     

Mononuclear cell content of human solid tumors

The mononuclear cell content and mononuclear cell function of human solid tumors were determined. Twenty-eight primary, and 17 metastatic solid tumors, including 6 lymph node metastases, were obtained from 45 patients. Cells were released from the tumors by mincing, scraping, and sieving. Analysis of the cellular content was determined by light microscopy, and tumor lymphocyte function was determined by stimulation of blastogenesis with PHA, PWM, Con-A, MLC, and SLO. A median of 3.3 × 10⁷ tumor cells with 12.5% viability was obtained per gram of tumor, and a median of 7.8 × 10⁶ lymphocytes with a viability of 96% and 0.35 × 10⁶ macrophages with a viability of 85% was also obtained per gram of tumor. Significant lymphocyte responses to various mitogens and antigens were obtained in 31–40% of the tumor lymphocyte preparations. From 9.4% to 23% had vigorous responses with a stimulation index greater than 10. Primary tumors contained significantly more lymphocytes than metastatic tumors; thus, the percent lymphocytes in primary tumors was 13.5% compared to 5% for metastatic tumors. In contrast, some of the lymphocyte responses were significantly greater for metastatic tumors than primary tumors. These data suggest that the direct study of lymphocytes infiltrating primary and metastatic tumors may be a useful approach to understanding tumor immunity in man.     

Genetic analysis of childhood endodermal sinus tumors by comparative genomic hybridization

Childhood endodermal sinus tumors (CEST) are a distinct category of germ cell tumors that involve the testis and extragonadal sites of young children. Recurrent deletions of 1p and 6q have been reported by classic cytogenetic analysis of a small number of cases. Comparative genomic hybridization, a technique that screens the entire genome for genetic abnormalities, is applied to additionally define the genetic changes present in CESTs. Sixteen frozen CESTs (10 testicular, 6 extragonadal) obtained from Pediatric Oncology Group-affiliated institutions or from the Cooperative Human Tissue Network were analyzed. The most common changes were gain of 20q (10 tumors), 1q (6 tumors), 11q and 22 (4 tumors each), and loss of 6q (8 tumors with common deleted region of 6q24-qter), 16q (4 tumors), and 1p (4 tumors). Localized regions of gain were identified at 8q24 (2 tumors both showing c-myc amplification by fluorescence in situ hybridization). Gain of 12p, characteristic of adolescent germ cell tumor, was present in one testicular tumor. Comparative genomic hybridization was useful in defining genetic differences between adult and childhood tumors, in determining the common regions deleted on chromosome 6, and in identifying other involved loci to be correlated with clinical parameters in future studies.     

Fetal and Neonatal Cardiac Tumors

Primary tumors of the heart are uncommon in the fetus and neonate. Nevertheless, the widespread use of new imaging techniques has contributed significantly to earlier diagnosis, treatment, and thus improved survival. The clinical findings, imaging studies, pathology, and outcome of 224 fetuses and neonates with cardiac tumors collected from the literature are evaluated and discussed. Most tumors are benign, and of these rhabdomyoma is the most common, followed by teratoma, fibroma, oncocytic cardiomyopathy, vascular tumors, and myxoma. Malignant and metastatic tumors are described but are rare. Murmurs, arrhythmias, cyanosis, respiratory distress, and cardiac failure are the main presenting signs of cardiac tumors in the perinatal period. Disturbances in hemodynamic function are correlated with the size and location of the tumor. Cardiac vascular tumors have the best outcome, whereas malignant tumors have the worst. The purpose of this review is to concentrate on the fetus and neonate in an attempt to determine the various ways cardiac tumors differ clinically and morphologically in this age group from those occurring in older children and adults and to show that certain types of tumors have a better prognosis than others.     

Germ cell, mesenchymal, and thymic tumors of the mediastinum.

Germ cell, mesenchymal, and thymic tumors of the mediastinum are an uncommon and heterogeneous group of neoplasms. Together they account for less than 25% of mediastinal tumors in childhood. The majority of these tumors are found in the anterior and superior mediastinum, but germ cell tumors and mesenchymal tumors may be located in all compartments. They share a broad range of histological subtypes and clinical behavior, tendency to be large in size, and a requirement for complete surgical excision as the major requirement for successful therapy.     

Acute Pulmonary Embolism Resulting from Cardiac Tumors Associated with Tuberous Sclerosis

A case report of a neonate with cardiac tumors in association with tuberous sclerosis is presented. The rare complication of pulmonary embolism and its successful treatment by anticoagulation and thrombolytic therapy is described. The treatment of these cardiac tumors is conservative, because the tumors regress spontaneously when the diagnosis is made in infancy. However, embolic event may rarely occur as a complication of cardiac tumors. If the cardiac tumors are treated conservatively, it is important to pay close attention to the occurrence of pulmonary embolism during the follow-up period.     

Management of Childhood Brain Tumors: Consensus Report by the Pediatric Hematology Oncology (PHO) Chapter of Indian Academy of Pediatrics (IAP)

Brain tumors are the second most common childhood tumors and remain the leading cause of cancer related deaths in children. Appropriate diagnosis and management of these tumors are essential to improve survival. There are no clinical practical guidelines available for the management of brain tumors in India. This document is a consensus report prepared after a National Consultation on Pediatric Brain Tumors held in Delhi on 06 Nov 2008. The meeting was attended by eminent experts from all over the country, in the fields of Neurosurgery, Radiation Oncology, Pediatric Oncology, Neuropathology, Diagnostic Imaging, Pediatric Endocrinology and Allied Health Professionals. This article highlights that physicians looking after children with brain tumors should work as part of a multidisciplinary team to improve the survival, quality of life, neuro-cognitive outcomes and standards of care for children with brain tumors. Recommendations for when to suspect, diagnostic workup, initial management, long-term follow up and specific management of individual tumors are outlined.     

QUANTITATIVE HISTOLOGIC FACTORS FOR GROUPING CHILDHOOD SUPRATENTORIAL NEUROGLIAL TUMORS

The histologic heterogeneity of childhood supratentorial neuroglial tumors, when quantified, identifies relatively homogeneous subgroups for prognostic purposes and for assignment in clinical trials. Our sample consisted of supratentorial tumors in the Childhood Brain Tumor Consortium. The data consist of reliably identified histologic features and demographic, clinical, operative, and survival information. Factor analysis was used to identify uncorrelated "factors," each represented by a different combination of histologic features in 703 tumors. The defining histologic features were used to label each factor. The heterogeneity of each tumor was summarized using the factor scores for each factor. We compared the survival estimates of subgroups of tumors within common diagnostic classes. We identified five uncorrelated quantitative factors that accounted for much of the histologic variation. Our factor labels were Jumbo, Fibrillary, Proliferative, Spongy, and Oligodendroglial. Two thirds of tumors had high scores on two or more factors, indicating a high degree of heterogeneity among these tumors. Eighty-four percent of supratentorial tumors were accounted for by 19 nonoverlapping relatively homogeneous histologic groups. The five quantitative factors complement standard qualitative taxonomies by summarizing more completely the histologic feature aspects of a tumor than by diagnosis alone and quantify the histologicheterogeneity of individual tumors. Histologically homogeneous groups of tumors are essential for clinical trials, biologic research, and prognostic models.     

Malignant germ cell tumors in childhood

Malignant germ cell tumors are a very heterogeneous group of neoplasms that have historically carried a poor prognosis. A review of the cooperative studies on malignant extracranial germ cell tumors in children was performed to update the progress in treatment and outcome for this rare group of tumors. The advent of platinum-based chemotherapy in the 1980s dramatically improved the prognosis for these tumors and allowed redefinition of risk groups. Chemotherapy regimens have been sequentially modified to reduce toxicity while maintaining survival. Surgical management has evolved from an aggressive en bloc resection at diagnosis to a more tailored approach, with resection and meticulous staging for low stage tumors at diagnosis and biopsy with neoadjuvant chemotherapy for advanced stage tumors. Improved surgical margins and prognosis are seen in post chemotherapy resections for high stage tumors. Large persisting masses after chemotherapy are often due to coexisting elements of benign teratoma, and aggressive attempts at resection are warranted in these patients with expectation of successful outcome.     

Treatment of non-testicular germ cell tumors in children and adolescents with BEP and VIP: initial results of the MAKEI 89 therapy study]

The pilot protocol of the German Society of Pediatric Oncology for treatment of non testicular germ cell tumors was initiated in November 1987. The final protocol was started at 1. 1. 89. Different therapy was administered depending on histology, primary localisation or stage of tumors. Patients with malignant germ cell tumors such as dysgerminomas, embryonal carcinomas, yolk sac tumors or chorio carcinomas received BEP (Bleomycin 15 mg/m2/days 1-3, Etoposide 100 mg/m2/days 4-8, Cisplatinum 20 mg/m2/days 4-8), followed by VIP (Vinblastine 3 mg/m2/days 1 + 2, Ifosfamide 1500 mg/m2/days 1-5 including Mesna uroprotection and Cisplatinum 20 mg/m2/days 1-5). Patients with ovary tumors of stage 1 were treated with 3 courses of BEP, patients with ovary tumors stage II and extragonadal localisation received 3 courses of VIP in addition to 3 courses of BEP. In cases of extended tumors 4 courses of BEP were followed by delayed resection of tumors and 4 courses of VIP. Patients with intracranial germinomas were treated with 30 Gy of craniospinal radiation therapy and additional 15 Gy as a tumor boost. Since some cases of spinal extension were reported a spinal radiation therapy seems to be indispensable. Patients with intracranial embryonal carcinomas, yolk sac tumors or chorio carcinomas tumors were given 2 courses of BEP and VIP followed by 30 Gy of craniospinal radiation therapy and additional 20 Gy as a tumor boost. Patients with immature teratomas of the ovary grade 1-3 and grade 3 of tumors with extragonadal localisation were treated with 3 courses of BEP after resection of tumors. Until 1. 1. 1991 92 patients were reported to the study--27 with intracranial and 65 with extracranial primary localisation of tumors. 43 patients suffered from teratomas (including 20 immature teratomas grade 1-3), 18 from germinomas (seminomas/dysgerminomas) and 31 from malignant non-seminomatous germ cell tumors. After an observation period of 29 months disease-free survival rate was 80% (79/92 patients, Kaplan-Meier Statistics). Outcome of intracranial tumors was death or relapse in 2/9 patients with malignant non-seminomatous germ cell tumors, in 2/14 patients with intracranial germinomas, in 2/4 patients with teratomas. Patients with extracranial localisation of tumors suffered from death or relapse in 1/21 cases with non-seminomatous tumors, in 0/4 cases with dysgerminomas and 5/39 cases with teratomas. During pilot study one infant with a malignant non-seminomatous germ cell tumor died of a pneumopathia. Therefore infants treated according to the final protocol did not receive Bleomycin.(ABSTRACT TRUNCATED AT 400 WORDS)     

Tumors of the maxillofacial region in children: retrospective analysis and long-term follow-up outcomes of 90 patients

The aim of this study was to carry out a retrospective analysis of maxillofacial tumors in children and to present the long-term follow-up results. Our study was performed with a retrospective analysis of 90 patients under the age of 15 years with maxillofacial tumor treated in our clinic between 1985-2002. In addition, treatment modalities and long-term follow-up results of these patients were evaluated. According to our results, it was established that maxillofacial tumors were mostly observed in the 11-15 age group (39 cases, 43.3%) and on the mandible (48 cases, 53.3%). There were 21 (23.3%) odontogenic, 63 (70%) benign nonodontogenic and 6 (6.7%) malignant non-odontogenic. Mixed tumors were the most common type of the odontogenic tumors, and mesenchymal tumors were the most common non-odontogenic tumors. Surgical excision, curettage or en bloc resection were adequate for treatment of these tumors.     

Brainstem tumors: where are we today?

Brainstem tumors comprise 10-20% of all pediatric central nervous system tumors. The management of these tumors has evolved dramatically in the past century. Once considered uniformly fatal, it is now known that brainstem tumors have distinguishing characteristics and do not behave identically. The focality and location of the lesion is determined from the clinical history, presentation, and associated imaging. Based on these findings, it is possible to predict the behavior of the tumor and choose an appropriate intervention. Focal lesions have a good prognosis and are treated operatively while diffuse lesions have a poor prognosis and are managed medically. This article reviews the current classification of brainstem tumors, current management options and future directions for the treatment of these rare tumors.