Congenital tumors

  • 文章类型: Case Reports
    非免疫性胎儿水肿代表了多种疾病的终末期状态,包括转移性肿瘤.我们报告了一例非免疫性胎儿水肿,与超声检测到并通过磁共振证实的多个播散性回声结节病变相关。脊髓穿刺术显示贫血和血小板减少症。鉴别诊断包括组织细胞增生症X,急性白血病或转移性疾病。在第25+6周诊断出死产。尸检显示胎儿水肿,右肾上腺肿块,组织学上由突触素阳性的小圆形蓝色细胞组成的多个播散性结节,胎盘受累,与先天性未分化神经母细胞瘤M期一致的发现无相关染色体异常,MYCN和ALK基因也没有扩增异常。在与多发性结节性病变相关的非免疫性胎儿水肿的鉴别诊断中,应考虑转移性神经母细胞瘤。
    Non-immune hydrops fetalis represents the end-stage status of a variety of diseases, including metastatic tumors. We report a case of non-immune hydrops fetalis associated with multiple disseminated echogenic nodular lesions detected by ultrasound and confirmed by magnetic resonance. Cordocentesis demonstrated anemia and thrombopenia. Differential diagnosis included histiocytosis X, acute leukemia or metastatic disease. A stillbirth was diagnosed at week 25 + 6. The autopsy revealed hydrops fetalis, a right adrenal gland mass, multiple disseminated nodules histologically composed of small round blue cells positive for synaptophysin, and placental involvement, concordant findings with congenital undifferentiated neuroblastoma Stage M. No chromosomal abnormalities were associated, nor amplification abnormalities in MYCN and ALK genes. Metastatic neuroblastoma should be considered in the differential diagnosis of non-immune hydrops fetalis associated with multiple nodular lesions.
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  • 文章类型: Journal Article
    先天性肿瘤很少见,恶性先天性肿瘤并不常见。良性图,Mors可能会危及生命,取决于肿瘤的位置和大小。不同因素影响先天性肿瘤,如母体和胎盘激素和环境因素如药物,辐射,和感染。开发胎儿影像学检查方法和孕期连续随访是影响先天性肿瘤预后的重要因素。超声是用于胎儿评估的最常用方法。补充评估方法是MRI。这两种方法对先天性肿瘤的检测都很有帮助,并且广泛传播。这些成像方法帮助医疗团队做出合适的治疗决定。其中一些肿瘤自发消退,有些需要手术治疗.肿瘤的治疗发展迅速,最近已经使用了分子靶向药物。
    Congenital tumors are rare, and malignant congenital tumors are uncommon. Benign tu,mors might be life-threatening, depending on the location and size of the tumor. Different factors affect congenital tumors, such as maternal and placental hormones and environmental factors such as drugs, radiation, and infection. Developing fetal imaging methods and continuous follow-up during pregnancy are important factors in congenital tumor prognosis. Ultrasound is the most common method used for fetal evaluation. The complementary evaluation method is MRI. Both methods are helpful and widely spread for the detection of congenital tumors. These imaging methods help the medical team make a suitable decision about therapy. Some of these tumors regressed spontaneously, and some need surgical treatments. Treatment of tumors has developed rapidly, and recently molecular-targeted drugs have been used.
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  • 文章类型: Journal Article
    颅内肿瘤在生命的第一年是罕见的,在这个年龄段,是仅次于白血病的第二常见的儿科癌症。作为新生儿和婴儿中较为常见的实体瘤,它们表现出一些特点,例如恶性肿瘤的高发病率。常规超声检查更容易检测宫内肿瘤,但是由于缺乏或缺乏可识别的症状,诊断可能会延迟。这些肿瘤通常非常大且高度血管化。他们的移除具有挑战性,发病率和死亡率高于年龄较大的儿童,青少年,和成年人。它们在位置上也不同于年龄较大的儿童,组织学特征,临床行为,和管理。小儿低度神经胶质瘤占该年龄组肿瘤的30%,包括局限性和弥漫性肿瘤。其次是髓母细胞瘤和室管膜瘤。其他非髓母细胞瘤胚胎性肿瘤,以前被称为PNETS,通常也诊断为新生儿和婴儿。畸胎瘤在新生儿中具有表达性发病率,但逐渐下降,直到生命的第一年结束。免疫组织化学,分子,和基因组的进步正在影响对某些肿瘤治疗的理解和靶向,但是,尽管有这些进步,切除程度仍然是影响几乎所有类型肿瘤预后和生存的最重要因素.结果很难估计,5年生存率为1/4至3/4的患者.
    Intracranial tumors in the first year of life are rare and, in this age group, are the second most common type of pediatric cancer after leukemias. As the more common solid tumor in neonates and infants, they present some peculiarities such as the high incidence of malignancies. Routine ultrasonography made easier to detect intrauterine tumors, but diagnosis can be delayed due to the lack or scarcity of recognizable symptoms. These neoplasms are often very large and highly vascular. Their removal is challenging, and there is a higher rate of morbidity and mortality than seen in older children, adolescents, and adults. They also differ from older children with respect to location, histological features, clinical behavior, and management. Pediatric low-grade gliomas represent 30% of the tumors in this age group and comprise circumscribed and diffuse tumors. They are followed by medulloblastoma and ependymoma. Other non-medulloblastoma embryonal neoplasms, former known as PNETS, are also commonly diagnosed in neonates and infants. Teratomas have an expressive incidence in newborns but decline gradually until the end of the first year of life. Immunohistochemical, molecular, and genomic advances are impacting the understanding and targeting of the treatment of some tumors, but, despite all these advances, the extent of resection remains the most important factor in the prognosis and survival of almost all types of tumors. The outcome is difficult to estimate, and 5-year survival ranges from one-quarter to three-quarters of the patients.
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  • 文章类型: Case Reports
    婴儿肌纤维瘤(IM)是儿童的良性软组织肿瘤。它们是成纤维细胞-肌纤维母细胞起源的,并与其他梭形细胞肿瘤表现出相当大的形态重叠。这里,我们报告了两例单发肌纤维瘤,一个是新生儿,一个是2岁女孩。一名2天大的女孩出现严重的呼吸窘迫,并在插管期间死亡。尸检时,注意到累及口咽并延伸至喉部的肌纤维瘤。第二例是一名2岁女孩,硬腭有肌纤维瘤。IM必须与婴儿期和儿童期的其他良性和恶性梭形细胞肿瘤区分开来。在新生儿呼吸窘迫的鉴别中,应考虑口咽肌纤维瘤。
    Infantile myofibromas (IMs) are benign soft-tissue tumors of children. They are of fibroblastic-myofibroblastic origin and show considerable morphological overlap with other spindle cell neoplasms. Here, we present two cases of solitary myofibromas, one in a neonate and one in a 2-year-old girl. A 2-day-old girl presented with severe respiratory distress and died during intubation. At autopsy, a myofibroma involving the oropharynx with extension up to the larynx was noted. Second case was a 2-year-girl with a myofibroma in the hard palate. IM must be differentiated from other benign and malignant spindle cell tumors of infancy and childhood. Oropharyngeal myofibroma should be considered in the differentials of neonatal respiratory distress.
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  • 文章类型: Journal Article
    UNASSIGNED: Neuroblastoma can arise from extra-adrenal sites in the paraspinal sympathetic chain, including the presacral region, where they must be differentiated from an immature or malignant neural lesion arising from a teratoma.
    UNASSIGNED: We describe two congenital presacral neuroblastomas. The main clinical differential diagnoses were sacrococcygeal teratoma and meningomyelocele. Pathologically, they lacked teratomatous tissues, lacked germ cell serum markers, were localized without metastases, and were MYCN non-amplified. Both patients have done well without chemotherapy at 18 and 15 months of follow-up.
    UNASSIGNED: Congenital presacral neuroblastoma should be differentiated from teratomatous lesions, and in general have a good prognosis.
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  • 文章类型: Case Reports
    Rhabdoid tumor is a highly aggressive sarcoma found in young children that occurs in the kidney, central nervous system and soft tissue sites. Rarely, it presents in the fetus or neonate and is associated with a dismal prognosis. We report a case of a 28-week gestation fetus presenting with hydrops fetalis who died soon after delivery, found at autopsy to have a rhabdoid tumor of the thoracic cavity with placental metastases and provide a review of the literature of congenital sarcomas.
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  • 文章类型: Journal Article
    Neonatal tumors are different from tumors of the older children and knowledge gained from treating older children can not be extrapolated to neonates. Neonates have immature physiology and their haematopoietic and immune systems are not fully developed and the response to therapy is unpredictable. Hence it is imperative to study these tumors as separate entity. The aim of this study is to analyse this rare set of tumors in terms of their incidence, clinical features and management.
    All babies admitted in our hospital with tumors from January, 2011 to January 2016 were studied. Tumor-like conditions like haemangioma, lymphangioma and hamartomas were included. The age, sex distribution, type of tumor and management were studied.
    A total of 51 cases were registered out of which, 29 cases were haemangiomas and lymphangiomas. Of remaining 20 cases, 5 were benign ovarian cysts, 3 were neuroblastomas, 3 were congenital fibrosarcomas, 3 were sacrococcygeal teratomas. Wilm\'s tumor, congenital mesoblastic nephroma, haemangioendothelioma of liver and others formed the remaining six cases.
    Our study insists that the neonatal tumors are distinct subset of pediatric tumors, requiring careful selection of treatment modalities and most of the solid tumors can be successfully managed if diagnosed and treated early. Neonatal tumors are defined as tumors which are diagnosed before the first month of life. Some of them can be congenital (present at birth). Neonatal tumors are different from tumors in older children in terms of etiopathogenesis, behavior and response to therapy as well as long-term outcomes.
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  • 文章类型: Journal Article
    OBJECTIVE: The purposes of this article are to provide an up-to-date overview of neonatal soft-tissue tumors, including information regarding their unique nature, and to present practical imaging techniques and characteristic imaging findings.
    CONCLUSIONS: Neonatal soft-tissue tumors are a unique set of neoplasms that often have characteristic clinical and imaging findings. Imaging evaluation, mainly with ultrasound and MRI, plays an important role in the initial diagnosis, staging, preoperative assessment, and follow-up evaluation. Clear understanding of practical imaging techniques combined with up-to-date knowledge of characteristic imaging findings can help the radiologist provide a timely and accurate diagnosis of these neoplasms and can lead to optimal neonatal patient care.
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  • 文章类型: Journal Article
    The occurrence of congenital neuroblastoma presenting at birth with symptoms of epidural compression secondary to spinal canal invasion is rare. Almost all cases reported in the literature have survived from the tumor but suffer severe sequelae, with the exception of the 2 most recently described whose birth was anticipated. The 3 cases of this article have been followed for a minimum of 5 years with the aim to describe their definitive late complications. In none of these cases had the routine ultrasound scan performed in third trimester of pregnancy discovered a tumor mass, nor had it shown abnormal fetal movements. All had leg hypotonia detected on the first day of life. In all, both primary and intraspinal tumors responded well to chemotherapy. All survive with motor deficit and severe bladder dysfunction despite early physiotherapy. Scoliosis has developed in the case with the longest follow-up. The description of these patients enforces the importance of early diagnosis of tumor masses in late pregnancy. Neonatologists should be aware of this rare clinical entity and take it into account in the differential diagnosis with other conditions of early-onset hypotonia. On the other hand, obstetric sonologists should be aware of the possibility to detect such rare tumors in late pregnancy, as anticipation of delivery may reduce the risk of late sequelae.
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  • 文章类型: Case Reports
    Multiple synchronous tumors presenting in infancy raise concern for inherited or sporadic cancer predisposition syndromes, which include Beckwith-Wiedemann syndrome, familial adenomatous polyposis syndrome, and Li-Fraumeni syndrome. We report a case of a 7-month-old previously healthy male born following an in vitro fertilization-assisted twin pregnancy who presented with new-onset refractory shock, severe acidosis, and rapid decline over several hours. An autopsy revealed a ruptured liver involved by hepatoblastoma, an adrenal gland involved by neuroblastoma, and multiple cutaneous capillary hemangiomas. Standard genetic testing demonstrated that both twins were Gaucher disease (GD) carriers without evidence of other known cancer predisposition syndromes. This report describes a unique association of multiple synchronous tumors, which underscores the utility and importance of the pediatric autopsy. Moreover, given that the reported child was a GD carrier, the possibility the tumors were the result of a GD-mediated cancer-associated phenotype or an unrecognized sporadic clinical syndrome remains an unanswered, but intriguing, question worthy of further investigation.
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