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)

Two alpha-fucose-binding lectins, Ulex europaeus agglutinin I (UEA I) and Lotus tetragonolobus agglutinin, were employed to compare and contrast the distribution of fucosubstance in normal human kidneys and a variety of renal tumors. The study employed a total of 31 kidneys surgically removed for the presence of a variety of tumors, including 11 unilateral Wilms' tumors, two cases of bilateral Wilms' tumors, 13 renal cell carcinomas, two congenital mesoblastic nephromas, one renal oncocytoma, one neuroblastoma metastatic to the kidney, and one clear cell sarcoma of the kidney. The results show that UEA I-reactive fucosubstance is detected in vascular endothelium of all kidneys and tumors, except bilateral Wilms' tumors. The presence of UEA I-reactive alpha-fucose in the vasculature of unilateral but not bilateral Wilms' tumors defines a unique histochemical distinction between the two groups of tumors. Conceivably, this property might be exploited as a screening procedure for the more aggressive bilateral neoplasms. Other findings detail histochemical differences between UEA I and L tetragonolobus agglutinin, as evidenced by the ability of one lectin to stain a particular cell type that is not reactive with the other lectin.
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PMID:Distribution of fucosubstance in kidney and related neoplasms. Absence of lectin-reactive alpha-fucose from the vasculature of bilateral Wilm's tumors. 284 40

The records of 46 children treated with ifosfamide between 1987-1994 were analyzed. 24 received the drug as first-line chemotherapy in combination with vincristine and etoposide or with adriamycin, vincristine and cyclophosphamide. Complete remission was achieved in 8/10 children with soft tissue sarcoma, 7/8 with Ewing's sarcoma, 2/2 with ovarian carcinoma and 2/2 with clear cell sarcoma of the kidney. 22 children in whom first-line chemotherapy failed were then treated with ifosfamide and etoposide. Complete remission was achieved in 7/10 children with osteosarcoma, 1/2 with Wilms' tumor and 1/3 with neuroblastoma.
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PMID:[Ifosfamide in pediatric malignancy--experiences in the Northern Israel Oncology Center]. 876 70

In contrast to the occurrence of brain metastases advanced malignant tumours in adult cancer patients, the dissemination of solid tumours to the brains of paediatric cancer patients is very uncommon. We present a neuro-pathological and clinical study of a group of children and adolescents with brain metastases (BM) from extracranial solid malignancies. The analysed patients were diagnosed with soft tissue sarcomas (three), germ cell tumours (three), or osteosarcoma, neuroblastoma, clear cell sarcoma of the kidney, or pleuropulmonary blastoma (one each). In our series, BM frequently coexisted with pulmonary metastases. Three different metastatic patterns were discernible: a solitary tumour, multiple lesions and diffuse parenchymal dissemination. Two cases showed haemorrhagic presentation. Most of the children died due to BM progression, while children with germ cell tumours showed the best prognosis. The histopathological pictures of BM can be different from the primary tumour, showing dedifferentiation or a diverse neoplastic component. The autopsy examination can still be helpful in the final diagnosis of certain cases with atypical clinical presentations.
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PMID:Brain metastases in paediatric patients: characteristics of a patient series and review of the literature. 2221 17

The pathogenesis of pediatric malignant tumors is associated with congenital abnormalities. Oncogenes and antioncogenes are identified in some of these cases. Neuroblastoma arises from the adrenal medulla and sympathetic ganglia. Most neuroblastomas produce catecholamine. Urinary vanillylmandelic acid(VMA)and homovanillic acid(HVA), metabolites of catecholamine, are sensitive tumor markers. Risk stratification according to tumor stage and a combination of prognostic factors helps determine the appropriate therapeutic strategy in clinical settings. Nephroblastoma(Wilms tumor)is the most common pediatric renal tumor and is often accompanied by congenital anomalies. Surgical resection of the tumor and the involved kidney is the initial treatment recommendation in the US and Japan. Consecutive chemotherapy and radiotherapy are administered after surgical staging and a definite histopathological diagnosis. Prognosis is relatively good for most nephroblastoma cases with a favorable histology. In addition to nephroblastoma, clear cell sarcoma of the kidney, characterized by a tendency to metastasize to the bone, is a renal tumor with poor prognosis. Rhabdoid tumor of the kidney is another tumor type; however, its pathogenesis is still unknown and it is associated with extremely poor prognosis because of the lack of effective therapeutic measures. Hepatoblastoma is the most common malignant liver tumor. The serum alpha-fetoprotein level is the most effective tumor marker. Complete surgical resection of the involved liver lobe is the definitive approach for cure. Preoperative chemotherapy increases the possibility of complete surgical resection. High-risk patients have a poor prognosis.
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PMID:[Clinical features of solid malignant tumors in childhood]. 2386 21

Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm's tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/PNET have a specific t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient's condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.
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PMID:Primary primitive neuroectodermal tumor of kidney: a rare case report with diagnostic challenge. 2494 71