An extragonadal retroperitoneal endodermal sinus tumor--results of treatment and treatment sequelae in a 3-year-old boy

A case of an extragonadal endodermal sinus tumour in a three-year old boy is presented. Complete resection of the retroperitoneal tumour was possible after initial chemotherapy. Combined radio-chemotherapy after tumour removal led to complete remission now lasting for more than 5 years. There was unilateral renal atrophy and diverticulum of the stomach as treatment related complications. The favourable prognosis of this tumour due to intensified treatment is discussed in relation to treatment sequelae. The interdisciplinary approach in treatment of cancer in children is emphasized.     

Successful treatment of inflammatory myofibroblastic tumor with malignant transformation by surgical resection and chemotherapy

Inflammatory myofibroblastic tumor (IMT) is a tumor composed of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. The authors present the case of a 7-year-old boy with an abdominal mass diagnosed as IMT with malignant transformation. The tumor recurred twice after attempts at resection and was initially treated with vincristine and etoposide. After a third recurrence and incomplete resection, he was treated with cisplatin, Adriamycin, and methotrexate. He is disease-free after 2 years, representing successful combined surgery and chemotherapy in the treatment of malignant IMT. The use of chemotherapy for aggressive myofibroblastic tumors is reviewed.     

Intracranial and mediastinal inflammatory myofibroblastic tumour

Inflammatory myofibroblastic tumour (IMT) is a rare neoplastic condition, commonly known as inflammatory pseudotumour, which is the most frequent primary tumour of the lung in childhood. IMT can have an extrapulmonary location, but is infrequent in the brain and mediastinum, and it is extremely unusual that both lesions appear simultaneously as in our patient. The definitive diagnosis is established by biopsy, but there are some MRI findings that can be useful to identify the lesion. Received: 13 January 2000 Revised: 3 October 2000 Accepted: 20 October 2000     

Carcinoid and mucoepidermoid bronchial tumours in children

The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour and 6 cases of mucoepidermoid tumour. The mean age of the patients was 10.5±3.0 years, with a range of 5 to 15 years. Twelve and 6 patients presented with evidence of bronchial obstruction and haemoptysis, respectively. Fibre optic bronchoscopy confirmed the presence of a bronchial tumour in all cases and endobronchial biopsies were diagnostic in 11 of 12 cases. A chest CT scan revealed the presence of a hypervascular tumour in 8 of 12 patients. The distribution of the location of the tumours was equal between the right and the left lung, and, in 9 cases, the airways were totally occluded by the tumour. Complete surgical resection (lobectomy in 15 patients and pneumonectomy in 2 patients) was performed in all cases without pre-operative chemotherapy or radiotherapy. The mean duration of follow-up was 4.0±3.0 years. In 2 patients, auscultation assymetry and an episode of haemoptysis revealed the recurrence of a mucoepidermoid tumour, successfully cured by removal of the tumour and chemotherapy and radiotherapy in one child. No death was observed. Conclusion:Pulmonary carcinoid and mucoepidermoid tumours are rare in children. Bronchoscopic removal should not be performed. With aggressive surgical therapy, the prognosis is excellent. Fibre optic bronchoscopy confirms the presence of an endobronchial mass. A biopsy is needed for diagnosis and complete surgical removal is the treatment of choice. Long-term results are excellent but a clinical follow-up is recommended.No financial support was received for this study.     

A rare cause of anaemia associated with hypertension in a 14-year-old girl

We report a rare example of anaemia and hypertension due to an incomplete Carney triad in a 14-year-old girl with no previous medical history. This rare non-familial syndrome generally involves two disparate tumours: gastrointestinal stromal tumour, paraganglioma and/or pulmonary chondroma. The complete triad is a syndrome that involves at least five loci: stomach, oeophagus, lung, the paraganglionic system, adrenal (cortex or medulla). The pathogenesis is unclear as these tumours have different embryological origins. Surgical treatment is necessary, and long-term follow-up is advisable as patients with Carney triad may re-present with tumour(s), even several years after the first presentation.     

川崎病恢复期颈动脉内膜-中膜厚度变化及相关因素分析

目的 观察川崎病(KD)患儿恢复期颈动脉内膜-中膜厚度变化及相关因素,为防治KD血管病变提供科学依据.方法 收集30例2 ～ 4岁KD恢复期患儿及30例同年龄健康儿童,测定其颈动脉内膜-中膜厚度(IMT)、丙二醛(MDA)、白介素-1β(IL-1β)、γ-干扰素(IFN-γ)和体质指数(BMI).结果 KD患儿颈动脉IMT为(0.40 ± 0.03)mm,对照组为(0.37 ± 0.04)mm,两者比较差异有统计学意义.KD患儿MDA 为(2.56±0.18)nmol/ml,对照组为(2.09±0.24)nmol/ml,两者比较差异有统计学意义.两组间IL-1β、IFN-γ、BMI比较,差异均无统计学意义.直线相关分析显示,颈动脉IMT与MDA呈正相关(r ＝ 0.463,P ＜ 0.05),而与IL-1β、IFN-γ和 BMI水平均无相关性(P均＞ 0.05).结论 KD恢复期患儿颈动脉内膜-中膜增厚,与氧化应激有关.     

Body composition phenotypes and carotid intima-media thickness in 11–13-year-old children

Early detection of impairment in vascular structure is an important clinical pursuit. However, it is unknown which measure of body composition best predicts vascular wall changes. We assess the differences in body composition among intima-media thickness (IMT) tertiles and determined which measures of body composition are associated with IMT in 385 children aged 11–13 years (196 girls). In this cross-sectional study, body mass index (BMI), waist circumference (WC), body fat mass (BFM), and trunk fat mass (TFM) from dual-energy radiographic absorptiometry and IMT through high-resolution ultrasonography were collected. Differences in body composition measures among IMT tertiles [low IMT (LIMT), ≤0.46 mm; middle IMT, 0.46–0.53 mm; higher IMT (HIMT), ≥0.53 mm] were assessed with ANOVA/ANCOVA after categorization. Regression analysis was used to assess the relationships between body composition and IMT. The groups were similar for sex, age, and maturity (p?>?0.05). As compared with LIMT group, subjects with HIMT had higher mean values of BMI, BFM, TFM, and WC (p?<?0.05). Significant differences were found for WC even when controlling for BMI (p?<?0.05). Combining all subjects, IMT was significantly correlated to BMI, BFM, TFM, and WC (p?<?0.05). In multiple regression, WC was the only predictor of IMT (β?=?0.22, p?<?0.001). Conclusion: Differences exist in body composition variables among IMT tertiles. In the overall model, WC was the only obesity-related predictor of increased IMT in 11–13-year-old children.     

Evaluación del grosor del complejo íntima-media de la carótida en la hipercolesterolemia familiar durante la infancia

IntroductionFamilial hypercholesterolemia (FH) is characterized by exposure to severely elevated LDL-cholesterol from birth, which produces lipid deposits, which can be measured by means of intima-media thickness (IMT).Subjects and methodsThe IMT and concentrations of cholesterol and its fractions, triglycerides, alipoproteins Apo-A1, Apo-B and endothelial risk factors (homocysteine and high sensitivity protein C ) were determined in 89 patients (44 males) from 2 to 19 years (9.54±3.91 years). IMT was measured by ultrasound using a 12 MHz linear array transducer in both carotids to 1 cm of the bulb. The IMT mean was compared with age, sex and analytical parameters using multiple regression analysis.ResultsThe mean values were: IMT 0.334±0.088 mm, total cholesterol 273.62±91.93 mg/dl, LDL-cholesterol 204.21±86.16 mg/dl, LDL/HDL 3.83±1.45, apoprotein A1 134.61±26.49 mg/dl, apoprotein B 130.59±40.59 mg/dl, homocysteine (median) 7.16 mmol/dl, Protein C (median) 0.3 mg/l. Using multiple regression analysis, only age was associated with IMT (P=0.049), a mean 0.005 mm (95% CI: 0.000–0.010) being the annual increase: up to 12 years the increase in IMT was 0.002 mm/year on (95% CI: ?0.007–0,010) and then from that age it was 0.013 mm/year (95% CI: ?0.023–0.049).ConclusionsThe measurement of the carotid IMT could become an objective parameter in the evaluation of the FH in childhood. In our study, it is only associated with age, the increase being most marked from 12 years onwards.     

Early onset of subclinical atherosclerosis in young persons with type 1 diabetes

OBJECTIVE: To evaluate the degree of atherosclerosis and its risk factors in adolescents and young adults with type 1 diabetes. STUDY DESIGN: We measured carotid artery intima-media thickness (IMT) in 142 subjects with type 1 diabetes (mean [SD] age = 16.0 [2.6] years) and 87 control subjects (18.8 [3.1] years). Fasting lipid and homocysteine levels, degree of glycemic control, blood pressure, and body mass index were measured in persons with diabetes. RESULTS: The mean carotid IMT was greater in persons with diabetes ( P = .002). Among subjects with type 1 diabetes, lipid levels were significantly higher in female subjects compared with male subjects. The mean carotid IMT was significantly higher in persons with a diabetic complication (including hypertension, retinopathy, or microalbuminuria). In male subjects but not female subjects, HDL cholesterol and the LDL/HDL ratio were correlated with carotid IMT. CONCLUSIONS: Adolescents with type 1 diabetes have increased atherosclerosis compared with control subjects. Risk factors for increased carotid IMT in these younger patients include diabetic complications and HDL cholesterol and the LDL/HDL ratio, which may be sex-specific.     

Small bowel intussusception by local recurrence of an inflammatory myofibroblastic tumor: report of a case and review of the literature

Inflammatory myofibroblastic tumor (IMT) of the ileum is a rare, usually solitary lesion, that frequently presents small-intestinal intussusception and obstruction. We describe an IMT of the ileum in a 4.5-year old child who presented a small bowel intussusception. During laparotomy, an annular mass around the ileum was resected, and the IMT was histologically diagnosed. Three months after the operation, the patients were hospitalized with the symptoms of intestinal obstruction. Laparotomy showed a ileal intussusception. Along the previous suture line of anastomosis, a smooth polypoid tumor was evident. Segmental resection of the ileum, including the tumor mass, was performed. The IMT was immunohistochemically diagnosed. The patient was asymptomatic at 3 year follow-up. A review of the literature for this rare entity emphasizes the importance of immunohistochemical confirmation of its benign nature. Because of the risk of local recurrence, IMT cases should have a long-term follow-up.     

Intravascular Inflammatory Myofibroblastic Tumors in Infancy

Inflammatory myofibroblastic tumor (IMT), previously described as inflammatory pseudotumor, can occur at any age but is a recognized soft tissue tumor of childhood. Less than 10 previous cases have been described of IMT affecting the heart, in patients ranging from 5 months to 17 years of age. We present three unusual, but similar, cases of IMT in infants, which were all predominantly intravascular in location, one of which was associated with death due to angiodestructive lesions of the coronary and cerebral arteries. These cases demonstrate an apparently distinct phenotype, with a predominant intravascular location of the tumor. Furthermore, this series highlights the difficulty in categorizing such lesions as benign versus malignant on histological grounds alone. IMT should be considered in the differential diagnosis of unusual pediatric intravascular spindle cell lesions.     

Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor (IMT) is an uncommon solid tumor that was originally described in the lung. A 4‐year‐old girl was admitted to hospital with urticarial rash. On chest radiographs, an opacity was seen in the inferior zone of the left lung, and computed tomography showed a mass in the left lower lobe. Left lower sleeve lobectomy was performed, and the diagnosis was confirmed as IMT. Sleeve resection is the best option in lesions located in the mainstem bronchus or secondary carina. Herein, we present a rare case of IMT of the lung that was successfully treated with sleeve lobectomy. There have been fewer than 15 childhood cases of IMT reported in the literature, and the present 4‐year‐old patient is one of the youngest.     

Pediatric inflammatory myofibroblastic tumor: anaplastic lymphoma kinase (ALK) expression and prognosis

BACKGROUND: Pediatric inflammatory myofibroblastic tumor (IMT) is rare, with unpredictable clinical behavior. Recently, it has been associated with anaplastic lymphoma kinase (ALK) expression. METHODS: Patients under age 16, treated for IMT between 1976 and 2000 were reviewed. Mean follow-up was 8 years (range 1 month-22 years). RESULTS: Eight children had IMT, with a mean age of 6 years (range, 11 months-14 years) and female to male ratio of 3:1. Tumor location was lung (four patients), abdomen (two patients), lung and abdomen (one patient), and abdomen, head, and neck (one patient). Presenting symptoms included anemia (seven patients), fever (six patients), and dyspnea (four patients). Laboratory results included thrombocytosis (six patients), hypergammaglobulinemia (four patients), elevated sedimentation rate (four patients), and leukocytosis (three patients). Immunohistochemistry revealed ALK expression in four of eight tumors. Four children had complete resection and are alive. Two of these children had ALK-positive tumors. Four patients had incomplete resection, and two had recurrences treated successfully with resection and radiotherapy; the other two died of disease. For the incomplete resection patients, those that were ALK-positive lived, and those that were ALK-negative did not. CONCLUSIONS: Eight children were treated for IMT over a 15-year period. ALK expression was found in half the tumors. Prognosis was improved with ALK expression and complete surgical resection.     

Abdominal obesity is an independent risk factor for increased carotid intima- media thickness in obese children

We aimed in this study to investigate carotid intima-media thickness (IMT) in obese children and evaluate the relationship of IMT to various cardiovascular risk factors. One-hundred four obese children (9.3 +/- 2.5 years) and 30 healthy age-matched control subjects were enrolled in the study. All children were assessed for fasting levels of glucose, insulin, lipid profile, skinfold thickness (SFT), waist circumference (WC), and blood pressure (BP). Insulin resistance was estimated by the homeostasis model assessment (HOMA) index. Carotid IMT measurements and non-alcoholic fatty liver disease (NAFLD) were diagnosed with ultrasonographic findings. IMT was significantly higher in obese children compared to controls (0.49 +/- 0.05 vs. 0.40 +/- 0.02 mm, p < 0.001). Significant positive correlations were found between increased carotid IMT and body fat percentage (BFP), body mass index (BMI), age, height, systolic BP, WC, SFT, triglyceride and insulin levels, and insulin resistance index. In a linear logistic regression analysis, the only parameter affecting the increase in carotid IMT was WC (beta: 0.589, p < 0.001). Furthermore, IMT was increased significantly in obese children with NAFLD when compared to obese children without NAFLD (0.54 +/- 0.04 vs. 0.48 +/- 0.05 mm, p < 0.001). Children with abdominal obesity are at increased risk for atherosclerosis, and WC can be used to determine the atherosclerosis risk in obese children.     

Is glycogen storage disease 1a associated with atherosclerosis?

Deficiency of microsomal glucose-6-phosphatase in liver and kidney leads to glycogen storage disease type la (GSD 1a). Notwithstanding intensive dietary therapy, moderate to severe dyslipidaemia and microal-buminuria, both known atherosclerotic risk factors, remain present. Although more patients reach adult age, no information is still available about accelerated athero-sclerosis. the aim of our study was to investigate whether GSD 1a was associated with premature atherosclerosis. In nine adolescent patients (mean age 22.7±3.4 years) and nine matched healthy control subjects, lipid profile, blood pressure, ankle-brachial indices, aortic distensibility and intima-media thickness (IMT) of the carotid and femoral arteries were determined. As expected, lipid profiles were significantly unfavourable in the patient group compared with the control group. No differences were found in blood pressure, ankle-brachial indices and aortic distensibility between both groups. IMT segments were comparable in both groups, with even thinner segments in the patient group. In different multivariate models, GSD la remained an independent predictor for a thinner IMT (R²=0.90; \=−0.69;P=0.018).Conclusion: glycogen storage disease type 1a is not associated with premature atherosclerosis, despite the existence of longstanding dyslipidaemia and microalbuminuria. Published online: 2 July 2002     

Assessment of brachial artery reactivity,carotid intima-media thickness,and adhesion molecules in pediatric solid tumor patients treated with anthracyclines

The aim of this study was to determine subclinical atherosclerosis and endothelial functional disturbance with measurement of carotid intima-media thickness (IMT), brachial artery reactivity (BAR), and levels of serum adhesion molecules in children with solid tumors who were treated with anthracyclines and are actually in complete remission. Fifty patients who were in remission and 30 healthy children were included in the study. Mean ages of patient and control groups were 13.5 ± 4.7 years (range: 3–23 years) and 12.00 ± 4.3 years (range: 4–21 years), respectively. The patients were divided into 3 groups according to cumulative doxorubicin dose: Group 1, ≤100 mg/m²; Group 2, 101–299 mg/m²; Group 3, ≥300 mg/m². The BAR and carotid IMT were measured in order to determine the endothelial function. The serum adhesion molecule levels in our patients and controls were also measured. The BAR of the patients with cumulative anthracycline dose ≥300 mg/m² was significantly lower than the patients with cumulative anthracycline dose ≤100 mg/m² and healthy controls (P =.005 and P =.003, respectively). Also, there was a negative correlation between brachial artery reactivity and increasing cumulative anthracycline dose (r = ?.287, P =.044). We also found significant difference between the mean carotid IMT of the patients and the healthy children (P =.041). No statistically significant difference was detected between the serum levels of sICAM-1 (soluble intercellular adhesion molecule-1), sVCAM-1 (soluble vascular cell adhesion molecule-1), sE-selectin of the patients and controls. The use of anthracyclines in pediatric patients with cancer could result in increase of the carotid IMT and endothelial dysfunction.     

Clitoromegaly and abdominal tumour as leading signs of a mediastinal non-Hodgkin lymphoma

A 2-year-old girl was admitted with clitoromegaly, mediastinal tumour and enlarged kidneys. A seventh cranial nerve palsy was associated with an increased cell count in cerebrospinal fluid. The diagnosis of a lymphoblastic lymphoma was confirmed by a cervical lymph-node biopsy.Six years after diagnosis and treatment according to the BFM protocol the girl remains in first complete remission.     

Inflammatory myofibroblastic tumor of the lung in children: anaplastic lymphoma kinase (ALK) expression and clinico-pathological correlation

The inflammatory myofibroblastic tumor (IMT) is a rare neoplastic lesion with a high incidence in children and young people, and may arise in lungs, soft tissue, or viscera. It is recognized as a borderline tumor with the possibility to recur, undergo malignant transformation, and metastasize. IMT is composed of fascicles of bland myofibroblastic cells admixed with an inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. We reviewed pulmonary IMT diagnosed at Garrahan Hospital in Buenos Aires, Argentina, during 12 years and examined the clinical, laboratory, and pathological features as well as molecular genetics. Eight pediatric cases were evaluated with a male-to-female ratio of 5:3 and a median age of 6 years at diagnosis. The most common lung localization was the upper lobe. All cases underwent surgical excision and no local recurrences were found. Five out of eight patients, including two cases with metastatic/multifocal lesions in the central nervous system (CNS), are alive and disease free after a median follow-up of 30 months. Anaplastic lymphoma kinase (ALK) expression was negative in all pulmonary samples by immunohistochemistry (IHC), however, rearrangement for ALK locus by fluorescence in situ hybridization was found in one lung and in two CNS samples. These findings may reflect higher sensitivity of the molecular biologic procedure compare to traditional IHC practice. In our pediatric experience, 25% of patients with lung IMT developed CNS lesions; therefore we consider that CNS screening in these patients should be considered, at diagnosis and later during follow up.     

Failure to thrive as a main symptom of intracranial tumors in early childhood

In the course of only three years we studied three children, who presented for a long time with failure to thrive as the only symptom of an intracranial tumour. Up to 24 months elapsed from the first manifestation at the age of four, ten and eleven months to the final diagnosis. A suprasellar tumour was discovered once, an infratentorial one twice. In spite of lower caloric intake a more rapid weight gain was documented after neurosurgery. The intracranial tumour is an important part of differential diagnosis in infants and young children not thriving satisfactorily.     

胶囊内镜在儿童小肠疾病诊治中的应用价值

目的 对疑有小肠疾病的儿童进行胶囊内镜检查,评估胶囊内镜在儿科的应用价值以及安全性.方法对2004年6月-2008年6月疑有小肠疾病的住院和门诊儿童43例进行胶囊内镜检查,男28例,女15例,年龄6～18岁,体重15～60 kg,身高110～180 cm,观察胶囊内镜检查成功率和失败率、胶囊内镜通过胃、小肠的平均时间,病变检出率和与最终诊断的符合率,操作过程中患者的耐受性和并发症;由胶囊内镜传送图像的质量评价;禁食8 h后检测时小肠的清洁度.结果 所有患儿均顺利吞服胶囊,检查期间耐受性好.共进行胶囊内镜检查46例次,成功43例次,失败3例次(成功率94%);胶囊内镜通过胃的平均时间为73(3～600)min,小肠内平均运行时间为246(73～413)min;检出病变37例(90%),与最终诊断符合的为31例(84%),出现并发症1例(2%);禁食8 h,小肠的清洁度佳,胶囊内镜所获取图像质量良好.结论 对于儿童不明原因的小肠疾病,特别是不明原因的小肠出血和小肠克罗恩病的诊断,胶囊内镜是一种安全和有效的检查方法.