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)

Patients with disseminated neuroblastoma are considered a poor-risk category, hence, our approach towards their treatment should be reconsidered in terms of the unique clinical and biological characteristics of neuroblastoma tumor growth. To this end, we have devised a treatment program consisting of surgery, and a schedule of sequentially escalating doses of cyclophosphamide combined with other drugs until a minimal disease status is obtained. When this is achieved, the patient is treated with maximal therapy, i.e., total body irradiation, high-dose L-phenylalanine mustard and dianhydrogalactitol followed by reconstitution with an autologous bone marrow graft. Details of this program include problems associated with evaluation of response, i.e., evaluation of risk, determination of minimal tumor burden, avoidance of toxicity, and compensation for supportive measures during maximal therapy. Additional problems of purging bone marrow of tumor cells are considered.
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PMID:Autologous bone marrow transplantation. A maximal therapy design for disseminated neuroblastoma. 389

We report an unusual case of progressive disseminated neuroblastoma occurring in a child with a family history and stigmata of von Recklinghausen's disease. A review of the literature confirms the extreme rarity of finding these two neurocristopathies in a single individual and thus undermines the widely held notion of an association--genetic or otherwise--between these two entities. We propose that synchronous neuroblastoma and von Recklinghausen's disease is accounted for by chance alone and therefore represents a randomly occurring phenomenon.
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PMID:Synchronous neuroblastoma and von Recklinghausen's disease: a review of the literature. 391 92

Total-body scintigraphy with 131I-meta-iodobenzylguanidine (131I-MIBG) was performed in 20 patients with neuroblastoma. In patients who were in complete remission no pathological concentration of 131I-MIBG was found. In 16 patients with residual, recurrent or metastatic neuroblastoma the tumor localizations were correctly identified by 131I-MIBG-scintigraphy In 5 patients additional tumor sites were found. In most patients an inverse relationship between myocardial and tumoral concentration of 131I-MIBG was noted. Dosimetric assessment of 131I-MIBG uptake in patients with metastatic disease revealed cases with considerable tracer concentration and long effective half lives in tumor localizations. It is concluded that 131I-MIBG total-body scintigraphy is useful in the diagnosis and follow-up of neuroblastoma and that therapeutic use of this agent is feasible, if patients are selected upon the merits of dosimetry.
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PMID:Total-body scintigraphy with 131I-meta-iodobenzylguanidine for detection of neuroblastoma. 391 23

The phenomenon of familial neuroblastoma is discussed in the context of case reports describing disseminated neuroblastoma in two of three half-brothers who share a common unaffected mother and who each have a different father. This family's cytogenetics proved to be unremarkable; also, the mother's peripheral blood DNA did not show tumorigenic capacities in transfection-nude mice experiments. An analysis of reported cases permits an updated examination of the clinical features of this entity and defines the limits of genetic counseling of families of all neuroblastoma patients. Multiple primaries are a hallmark of familial neuroblastoma. Most diagnoses are made in the first 18 months of life and at ages that fall within 12 months of the age of diagnosis of the other affected family member. Difficulties in determining the incidence and penetrance of an inherited susceptibility to neuroblastoma derive from undiagnosed tumors that have undergone regression or spontaneous maturation to benign ganglioneuroma, as well as from early deaths or long-term treatment complications that preclude reproduction and multigenerational pedigrees. Nevertheless, the risk of neuroblastoma in siblings or offspring of the large majority of persons with neuroblastoma appears to be less than 6%. Recent observations concerning chromosomal aberrations and oncogenes in embryonal malignancies are presented in an integrated model of tumorigenesis that corresponds to clinical experience.
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PMID:Familial neuroblastoma. Case reports, literature review, and etiologic considerations. 395 26

The case of a 41/2-year-old girl with disseminated neuroblastoma and concomitant osteomyelitis is reported. Neuroblastoma was detected in the vertebral column, the right suprarenal fossa, the left side of cranium and in bone marrow aspirates. Osteomyelitis was present in the left femur and was due to Salmonella tennessee. For the first time in the literature the two lesions were demonstrated by means of simultaneous 131I-MIBG and 99mTc-DPD scintigraphy. The diagnoses were proved by direct histological and microbiological studies.
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PMID:Coincidence of infectious osteomyelitis and disseminated neuroblastoma: a diagnostic dilemma solved by scintigraphic imaging. 401 73

Proto-oncogenes represent a group of eukaryotic genes whose activated forms are implicated in the development of cancer. We have recently identified a human gene, N-myc, that is distantly related to the proto-oncogene c-myc. N-myc is expressed at abnormally high levels consequent to amplification in numerous human neuroblastoma cell lines and metastatic neuroblastoma tumours. In addition, enhanced expression of N-myc, often a result of amplification, has been found in retinoblastoma cell lines and tumours (refs 5, 7 and M.S., unpublished data) and in cell lines derived from small-cell carcinomas of the lung. Here, we show that enhanced expression of N-myc subsequent to co-transfections of an N-myc expression vector and the mutant c-Ha-ras-1(EJ) (from the human bladder carcinoma cell line EJ) is a factor in tumorigenic conversion of secondary rat embryo cells. The transformed cells elicit tumours in athymic mice and isogeneic rats. The ability of N-myc to contribute to neoplastic transformation of cultured mammalian cells raises the possibility that enhanced expression consequent to amplification of N-myc may be a factor in the aetiology of human neuroblastoma.
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PMID:Human N-myc gene contributes to neoplastic transformation of mammalian cells in culture. 404 Feb 14

Although rarely reported, neuroblastoma in monozygotic siblings merits attention because study of its features may aid in elucidating mechanisms of tumorigenesis. We describe disseminated neuroblastoma in one of monozygotic triplets, including both documentation of monozygosity and long-term follow-up of the unaffected co-twins. Information from reports of monozygotic twins concordant and discordant for neuroblastoma reinforces the hypothesis that hereditary factors may be predominant in neuroblastoma diagnosed in infants, whereas nonheritable random mutational genetic events may be more important in neuroblastoma diagnosed after infancy.
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PMID:Monozygotic siblings discordant for neuroblastoma: etiologic implications. 404 Sep 63

We have experienced 30 patients with neuroblastoma since 1975. Disseminated intravascular coagulation (DIC) developed in six of these patients. They were all in stage IV, namely disseminated neuroblastoma. These six cases with DIC proved that some advanced neuroblastomas have the potential to cause coagulopathy in the process of the disease. The plasma concentration of heparin was measured in some patients who were treated with heparin. The data revealed that the conventionally used intravenous heparin dose is not appropriate in the case of DIC. Effective treatment requires monitoring of the plasma concentration of heparin.
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PMID:Advanced neuroblastoma and disseminated intravascular coagulation: report of six cases. 405 31

We describe, in a young boy, a case of neuroblastoma presenting only as a painless unilateral testicular mass. Clinical and laboratory evidence of disseminated neuroblastoma was first apparent nine months after high orchiectomy. As far as we are aware this is the only reported instance of testicular neuroblastoma in the absence of disseminated disease. Discussion is directed to the possible explanations for this rare testicular lesion, and in addition, underlines the potential difficulties in the diagnosis of certain small cell tumors.
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PMID:Testicular neuroblastoma. 407 1

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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