Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

ICO - 10 (Thy-1 antigen) and K 20 (gp 120/200) monoclonal antibodies proved suitable for immunophenotyping solid tumors. The following typical antigen combinations were identified with regard to reaction with those antibodies: ICO - 10+ K 20- (neuroblastoma, malignant schwannoma, neurosarcoma and soft tissue sarcomas); K 20+ ICO - 10- (cancer of the tongue and esophagus, adenoma of the adrenal cortex, adenocarcinoma of the stomach, hepatoblastoma and nephroblastoma); ICO - 10+ K 20+ (immature teratoma and cervical and esophageal leiomyoma) and ICO - 10+ K 20- (malignant fibrous histiocytoma).
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PMID:[ICO-10 (Thy-1) and K-20 (gp 120/200) monoclonal antibodies in immunophenotyping of solid tumors in man]. 233 Jun 81

A 2-year-old boy presented with sexual precocity secondary to a hormone-producing hepatoblastoma. The tumor demonstrated an aggressive histologic pattern and stained immunohistochemically for alpha-fetoprotein and beta-subunit human chorionic gonadotrophin. After a successful liver transplant these tumor markers were closely followed. We conclude that histologic subclassification of hepatoblastoma is most useful for predicting likelihood of resectability. Tumors with predominantly fetal-type histology tend to be better circumscribed and are more likely to be resectable with improved survival relative to tumors with predominantly embryonal histology. The presence or absence of mesenchymal elements does not seem to have prognostic importance. Anaplastic features predict a poor outcome, but it remains for these to be uniformly defined and applied. Small uniform cellular histology with neuroblastoma-like differentiation appears to be particularly ominous. Serologic tumor markers, especially alpha-fetoprotein (AFP) and human chorionic gonadotrophin (HCG) are useful as monitors of potential tumor recurrence, but their efficacy is compromised by the unpredictable variation of tumor cell metabolism, undoubtedly a result of the genetic instability and heterogeneity of proliferating malignant cells.
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PMID:Hepatoblastoma presenting as isosexual precocity. The clinical importance of histologic and serologic parameters. 243 80

From 1975 to 1987, we have operated 20 cases with lung metastases. They were 12 females and 8 males with ages between 1,6 and 12 years old (average of 5 years). The histology of the primary neoplasia was: 13 cases with Wilms' Tumors, 3 cases with Ewing's Sarcoma and 1 case with Neuroblastoma, Rhabdomiosarcoma, Hepatoblastoma and Neuroectodermic Tumor of Askin. We found 38 metastases and there were performed through a Thoracotomy 28 wedge resection, 7 pulmonary lobectomies and 3 biopsies. The operative mortality was 0%. The most frequent location of the lesion were on medium and lower lobule of the right lung. The survival was 70% (14 cases) after 4 years of average of the follow up. The survival for Wilms Tumors was 61% (8 cases) and all patients with Ewing's Sarcoma, Rhabdomiosarcoma, Neuroblastoma and Neuroectodermic Tumor of Askin were alive. The Thoracotomy is a useful method for therapy of tumors in children with lung metastases associated to chemotherapy and radiotherapy.
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PMID:[Surgery of lung metastasis in childhood]. 248 69

Twenty-nine cases of solid malignant tumours in paediatric age group were studied. The incidence of different tumours in order of frequency were lymphomas (37.9%), Wilms' tumour (24.1%), neuroblastoma (17.2%), soft tissue sarcoma (10.4%) and rare tumours (10.4%). Maximum number of tumours occurred in 1-5 years' age group. Male predominance was noted (male:female as 2.2:1). Among the lymphomas, non-Hodgkin's lymphoma outnumbered Hodgkin's lymphoma. Left kidney affection was seen more than the right kidney among cases of Wilms' tumours. Neuroblastomas presented commonly as abdominal masses. All the 3 cases of soft tissue sarcomas were embryonal rhabdomysoarcomas. Rare tumours encountered in the study were ovarian tumours (arrhenoblastoma), endodermal sinus tumours and hepatoblastoma.
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PMID:Pattern of paediatric solid malignant tumours in southern Orissa. 258 29

Developments in the treatment of childhood cancer have been evaluated in patients who had been treated in the National Children's Hospital from 1965 to 1987. The total number of patients was 867, of which leukemia accounted for 376, malignant lymphoma 61, neuroblastoma 174, Wilms' tumor 55, yolk sac tumor 29, rhabdomyosarcoma 36 and hepatoblastoma 30. Patients were divided into three time intervals: the 1960s, 1970s and 1980s. A marked improvement in five-year survival was recognized in Wilms' tumor and yolk sac tumor, amounting to 80%, followed by rhabdomyosarcoma, acute lymphoblastic leukemia and malignant lymphoma. There was no improvement in patients with acute non-lymphoblastic leukemia, neuroblastoma and hepatoblastoma. Prognostic factors for neuroblastoma were further analyzed, and the age of onset and stage of disease were found to have remained constant for 23 years. Factors relating to the improvement of survival were discussed.
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PMID:Improvement in the treatment of childhood cancer: analysis of survival data from the National Children's Hospital (1965-1987). 284 93

Delayed primary operation has been started since 1982 in our department and 19 cases of solid tumor in childhood were treated with this method. They included 11 cases of advanced neuroblastoma, 5 cases of hepatoblastoma and 3 cases of yolk sac tumor in sacrococcygeal area. We discussed effectiveness of preoperative chemotherapy to the primary tumor and metastatic lesions, the best timing of operation after chemotherapy and the operative technique in delayed primary operation. Preoperative chemotherapy were useful in almost all cases of delayed primary operation, so the primary lesion and lymph nodes metastases were removed easily. We recommended it was the best timing of operation when 2 or 3 courses of preoperative chemotherapy were finished. CUSA (Cavitron Ultrasonic Surgical Aspirator) was the useful instrument to remove the primary tumor and lymph node metastases completely in advanced neuroblastoma preserving both kidneys.
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PMID:[Delayed primary operation of solid tumors in children]. 284 15

Serum neuron-specific enolase (NSE) was measured in 61 children at diagnosis with all stages of neuroblastoma. The median serum values for Stages I, II, III, IV, and IV-S were 13, 23, 40, 214, and 40 ng/ml, respectively. Mean serum levels were different between groups I versus IV, (P = 0.0004) II versus IV (P = 0.0001) and IV-S versus IV (P = 0.004). The prognostic value of serum NSE for disease-free survival was determined in 54 patients at risk for relapse 2 or more years after diagnosis. The disease-free survival rate of all patients with levels of less than 100 ng/ml was 27/34 (79%), whereas it was 2/20 (10%) for those with higher levels. In 28 patients with lower stage disease and a good prognosis (Stages I, II, and IV-S) NSE levels were not predictive of relapse. Only 1 of these 28 patients had a raised level (greater than 100 ng/ml) and survived without relapse, whereas 4 patients who relapsed had serum NSE less than 100 ng/ml at diagnosis. In patients with Stages III and IV disease, a raised serum NSE level was associated with poor outcome: only 1/19 (5%) survived with NSE levels greater than 100 ng/ml, whereas survival was 5/8 (63%) with values below 100 ng/ml. Serial samples were analyzed on 17 patients; all 8 patients with initial NSE levels greater than 100 ng/ml achieved near normal levels during remission (median, 21 ng/ml). However, in only 4/10 patients studied at time of relapse, did the levels rise coincident with relapse. The sera of 47 patients with other forms of cancer and 19 siblings of cancer patients were at or near the normal limits (0-15 ng/ml), with three exceptions: acute lymphoblastic leukemia (286 ng/ml), hepatoblastoma (176 ng/ml), and primitive neuroectodermal tumor (105 ng/ml). Serum NSE is a useful marker for patients with advanced neuroblastoma in whom elevated levels were associated with a poor outcome; the raised NSE levels returned to near normal after therapy. In patients with Stage IV-S disease serum NSE levels were significantly lower than those in Stage IV despite their extensive tumor burden. Serum NSE estimation may confirm Stage IV-S status and suggest a more benign clinical course.
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PMID:Serum neuron-specific enolase in children with neuroblastoma. Relationship to stage and disease course. 300 99

DNA was extracted from formalin-fixed and paraffin-embedded tissues of 85 patients with pediatric malignant solid tumors which had been resected at surgery or obtained at autopsy during a 24-year period. The tumors examined included 25 rhabdomyosarcomas, 12 Wilms' tumors, 10 hepatoblastomas and 37 neuroblastoma group tumors. Neuroblastoma group tumors were subclassified into 25 neuroblastomas and 12 ganglioneuroblastomas among which 6 composite ganglioneuroblastomas were included. Sample blocks were selected from both tumors and normal tissues in the majority of cases. We were able to reliably detect N- and c-myc gene amplification in tumor DNA by dot blot-hybridization. The N-myc gene showed approximately from 3- to 500-fold amplification in 19 of 33 cases of stage IV neuroblastoma group tumor. All of these 33 patients had been intensively treated with chemotherapy and/or radiotherapy. The c-myc was amplified 8-fold in 1 case of rhabdomyosarcoma, but neither N-myc nor c-myc was amplified in any cases of Wilms' tumor or hepatoblastoma. We retrospectively examined the association among N-myc gene amplification, prognosis, and histologic subtype in 33 patients with stage IV neuroblastoma group tumors. The survival of the patients with N-myc gene amplification was shorter than that of the patients without amplification of N-myc (p less than 0.05). There was no significant difference in prognosis between the 2 histologic subtypes; neuroblastoma and ganglioneuroblastoma, and the cases of tumors with amplified N-myc showed shorter survivals for each subtype (p less than 0.05). In every case of neuroblastoma group tumor, the copy number of the N-myc gene was the same among primary site and multiple metastatic tumors, even when the lesions showed differences in histologic subtype like neuroblastoma and ganglioneuroblastoma.
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PMID:Retrospective study on amplification of N-myc and c-myc genes in pediatric solid tumors and its association with prognosis and tumor differentiation. 341 33

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

Unfavorable prognosis for children with malignant liver tumors, is caused not only because of late diagnosis in this children, but because of imperfet methods of treatment. These methods taken from adults oncology have to be adapted for pediatric patients in front of their differences in biology, pharmacodynamic reactivity and tumor-host relationships in developing organism. Some methods of treatment can be done the same way as in adults, but another (intraarterial treatment) can be used in children with much better results then in adults. Primary malignant tumors in children are hepatoblastoma and hepatocelullar carcinoma (minority) and metastatic tumors are nephroblastoma or neuroblastoma. That makes probably basic difference with liver tumors in adults, as well as absence of hepatic cirrhosis in children. 42 children with primary and metastatic liver tumors were treated by the author in Clinical Department of Pediatric Oncology, Institute of Mother and Child, Warsaw. 19 of them was given intra-arterial chemotherapy for unoperable primary tumors, 6--systemic chemotherapy for the same reason, 5--radiotherapy (all of them neuroblastomas) and 15 was submited to surgery-From this group 13 was operated radicaly by means of right extended or left lobectomy. Only in one case, middle lobectomy was done. 3 children operated radicaly was previously treated with intra-arterial chemotherapy and only after significant remission, surgery was done. As a result 6 children is RFS for more than 3 years. One of them exclusively grace to intra-arterial treatment. Experimental investigation done in monkeys shown no long term morphological and/or functional disturbances after long time hepatic infusion with chemotherapeutic drugs. In conclusion it is to state that surgery is the best method of treatment in primary liver tumors, some time preceded with intra-aterial chemotherapy that is safe for normal liver tissue. In metastatic neuroblastoma in liver, radiotherapy with systemic chemotherapy can give 60% of RFS (recurrent free survival).
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PMID:[Evaluation of the methods of treatment of children with liver neoplasms]. 609 70


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