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)

The clinical profile and outcome of neuroblastoma in 103 children, older than one-year is presented. 74 had Stage IV, 27 Stage III and one patient each had Stage I or II disease. Treatment included chemotherapy followed by surgical resection/debulking. Radiotherapy was administered to those with residual tumor. Chemotherapy consisted of OPEC (vincristine, cyclophosphamide, cisplatin and etoposide). The caretakers of 54 (52.4%) children either did not opt for or defaulted therapy, whilst 3 patients died before chemotherapy could be initiated. Of the remaining 46 patients, the tumor progressed during therapy in 19 (41.3%). Relapse of disease was documented in 22 (47.8%) cases. Merely 4 (8.7%) children are disease free for a period of 16.5+/-6.7 months. Majority of children presented with advanced disease and the outcome was dismal with conventional non-myloablative chemotherapy.
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PMID:Profile and outcome of neuroblastoma with convertional chemotherapy in children older than one year: a 15-years experience. 1831 Jul 93

Although PET imaging has been available for more than two decades, its use has greatly increased lately due to the advent of PET/CT, readily available sources of commercially supplied FDG, and mobile scanners. These features have enabled PET scanning to expand beyond select major university medical centers, with on-site cyclotron facilities to smaller institutions including free- standing children's hospitals. In these settings, imaging is generally limited to FDG, which suffices for most applications, with the majority of studies performed for tumor imaging. FDGI is being used for evaluation of many tumors in children, with its use in lymphoma being the most established. In lymphoma, it has proven quite useful in determining whether active tumor is present in residual masses following treatment, which may otherwise contain only residual fibrous tissue. For brain tumors, FDGI has some relation to tumor grade, although its more important role is distinguishing recurrent or residual tumor from the effects of treatment, particularly radiation necrosis. For neurological evaluation, interictal FDGI is helpful in localizing potential seizure foci for subsequent subdural EEG monitoring. Because of the relatively long uptake time of FDG, true ictal studies cannot be performed with FDG, and these remain within the domain of SPECT imaging of tracers such as Tc-99m HMPAO. Examinations utilizing radiopharmaceuticals other than FDG are significantly more limited in their availability and are limited to PET centers with on-site cyclotrons. However, these additional agents open the door to many additional studies, including more specific tumor-imaging agents of certain tumors such as neuroblastoma. Another neurotransmitter, F-18-fluoro-L-dopa, is of benefit outside of the central nervous system for evaluating infantile hyperinsulism. The use of PET/CT in children is increasing quickly, particularly FDGI imaging of tumors. It is expected that over the next few years, its role will become increasingly better defined.
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PMID:Positron emission tomography/computed tomography (PET/CT) in children. 1861 94

Several transcripts have been claimed to be clinically valuable for detecting minimal disease in neuroblastoma, but they have not been prospectively compared in a standardized manner. Tyrosine hydroxylase (TH), dopa decarboxylase (DDC) and GD2 synthase (GD2S) mRNAs were analyzed in 554 blood (PB) and bone marrow (BM) samples from 58 children with neuroblastoma. Samples from 44 children with other diseases served as controls. High transcript concentrations of TH, GD2S or DDC in PB or BM at diagnosis were associated with poor prognosis. TH in BM above median indicated worse outcome for a homogenous cohort with high-risk neuroblastoma (survival probability 91% for TH below median versus 33% for TH above median, p = 0.009). The number of children with localized neuroblastoma with increased results in PB did not differ between the three transcripts. In these children, all without morphologically detectable neuroblastoma in BM, the number of patients with elevated GD2S in BM at diagnosis was significantly higher than for the other transcripts (10/16 elevated, p = 0.012). GD2S was elevated in PB from 10/28 controls without neuroblastoma compared to 1/28 for TH and DDC (p < 0.001). In BM from these children GD2S was significantly elevated. We conclude that high expression of TH and DDC both in PB and BM corresponds to metastatic neuroblastoma at diagnosis, residual disease, and poor outcome. Children with high-risk neuroblastoma and low levels of TH in BM at diagnosis may be cured by current therapy. GD2S is less specific than TH and DDC mRNA for neuroblastoma detection in PB and BM.
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PMID:mRNAs of tyrosine hydroxylase and dopa decarboxylase but not of GD2 synthase are specific for neuroblastoma minimal disease and predicts outcome for children with high-risk disease when measured at diagnosis. 1881 38

Immature DCs were generated from the peripheral blood monocytes from five children with refractory solid tumors (Ewing sarcoma, synovial sarcoma, neuroblastoma) using GM-CSF and IL-4. These DCs were then pulsed with tumor-specific synthetic peptides or tumor lysates in the presence of the immunogenic protein KLH for 12 h. Pulsed DCs were administered subcutaneously every one or two weeks in an outpatient setting without any toxicity. In one patient with Ewing sarcoma, the residual tumor disappeared following autologous PBSCT and DC therapy, and a complete remission has been maintained for 77 months. In two patients with synovial sarcoma or with neuroblastoma, growth of the tumors was temporally suppressed for one and 10 months, respectively, followed by their exacerbation. A DTH response was detected against KLH in all five patients and against the tumor lysate in one patient. In the patients with a possible DC-mediated anti-tumor effect, the number of CD8(+) HLA-DR(+) lymphocytes and INF-gamma(+)CD8(+) lymphocytes increased and an elevation of the NK cell cytotoxic activity was observed during and/or after DC therapy. DC-based immunotherapy may therefore be a feasible, well-tolerated and promising approach in the treatment of children with refractory malignant tumors.
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PMID:Immunotherapy with autologous dendritic cells and tumor antigens for children with refractory malignant solid tumors. 1906 17

Neuroblastoma is the most common extracranial solid tumor malignancy of childhood. Although it is generally responsive to treatment, high risk cases of neuroblastoma frequently recur. The prognosis for relapsed cases is extremely poor despite aggressive therapy. The frequency of relapse and subsequent failure of further treatment has spurred the need to develop non toxic and more effective treatments for targeting residual tumor cells during the phase of minimal residual disease. Traditional cancer therapies are non-specific, leading to the destruction of normal, healthy tissues. Failure to induce specific tumor immunity may be due to several immunosuppressive factors. Primary amongst these factors are: lack of co-stimulatory molecules on the surface of tumor cells, the ability of the tumor to modulate immunity in a suppressive manner and the presence of an immunosuppressive microenvironment at the location of the tumor. Unfortunately, tumor tolerance impedes the ability to establish immunity to tumor antigens and overcoming this tolerance is essential to developing effective tumor immunity. Vaccine strategies that target host immune effector cells with synthetic oligodeoxynucleotides (ODNs) that contain unmethylated CpG motifs (CpG-ODNs) represent a novel approach to overcoming tolerance in cancer therapy. This approach enables biasing of host immunity toward a proinflammatory Th1 and thus anti-tumor response. The addition of immunogenic tumor specific antigen to the CpG-ODN vaccine may allow for specific targeting and killing of established tumors.
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PMID:CpG oligonucleotides for immunotherapeutic treatment of neuroblastoma. 1918 94

High-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) was applied to improve the prognosis of patients with high-risk stage 3 neuroblastoma. From January 1997 to December 2006, 28 patients were newly diagnosed as stage 3 neuroblastoma. Nine of 11 patients with N-myc amplification and 5 of 17 patients without N-myc amplification (poor response in 2 patients, persistent residual tumor in 2 and relapse in 1) underwent single or tandem HDCT/ASCR. Patients without high-risk features received conventional treatment modalities only. While 8 of 9 patients underwent single HDCT/ASCR and the remaining one patient underwent tandem HDCT/ASCR during the early study period, all 5 patients underwent tandem HDCT/ASCR during the late period. Toxicities associated with HDCT/ASCR were tolerable and there was no treatment-related mortality. While the tumor relapsed in two of eight patients in single HDCT/ASCR group, all six patients in tandem HDCT/ASCR group remained relapse free. The 5-yr event-free survival (EFS) from diagnosis, in patients with N-myc amplification, was 71.6+/-14.0%. In addition, 12 of 14 patients who underwent HDCT/ASCR remained event free resulting in an 85.1+/-9.7% 5-yr EFS after the first HDCT/ASCR. The present study demonstrates that HDCT/ASCR may improve the survival of patients with high-risk stage 3 neuroblastoma.
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PMID:High-dose chemotherapy and autologous stem cell rescue in patients with high-risk stage 3 neuroblastoma: 10-year experience at a single center. 1965 49

Primary liver tumors in children are rare with malignant hepatoblastoma being the most common neoplasm. In this report, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma. Pathological assessment on a pre-operative bioptical specimen showed an immature teratoid tumor with no area of hepatoblastic differentiation present. Histological and immunohistological examination of the resected tumor specimen additionally showed tumor areas of very different differentiation pattern intermixed with each other, namely areas of hepatoblastoma-typical and neuroblastoma-like morphology as well as areas of rhadomyosarcomatous differentiation.After chemotherapy the tumor size increased and an extended right hemihepatectomy was performed. Post-operatively, the general condition of the child improved and adjuvant chemotherapy was started two weeks later. 36 months after initial diagnosis the patient is healthy, in good general condition, and without any sign of residual tumor disease.Overall, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma and was designated as mixed hepatoblastoma and teratoma. Though mesenchymal tumor portions can occur within hepatoblastomas, most commonly osteoid or chondroid, our case is different as it presents a large spectrum of mesenchymal and epithelial differentiation pattern in most of the lesion.
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PMID:Mixed hepatoblastoma and teratoma of the liver in a 3-year-old child: a unique combination and clinical challenge. 1990 20

Neuroblastoma is the most common extracranial solid tumor in early childhood with a pluripotent neural cell origin. The disease often infiltrates the bone marrow, and these cases are candidates for flow cytometric diagnosis and disease follow-up, more so in atypical cases that are diagnostically challenging by other methods. Here, we report two neuroblastoma cases, one with characteristic neuroblastoma cells forming Homer-Wright rosettes found in the bone marrow sample by histological examination. This case showed the classical immunophenotype with CD81/CD56/CD117 positive and CD45 negative labeling and 80% bone marrow infiltration. Minimal residual disease measurement was performed in a regular fashion, and flow cytometry results showed good correlation with other disease markers. Analyzing 300,000 events provided a sensitivity level of 0.01%. The second case showed atypical cell morphology on histological examination, after which flow cytometric analysis was initiated. Atypical cells displayed CD81 and bright CD56 but CD117 negative immunophenotype, with a 28% bone marrow infiltration. In most cases the primary tumor can be identified, but in this particular case only bone marrow involvement could be detected using flow cytometry, as such making it a powerful tool for diagnosis and disease monitoring.
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PMID:Classical and atypical neuroblastoma--case reports. 2133 95

Resveratrol, a nontoxic polyphenol, has been shown to inhibit tumor growth in a xenograft mouse model of neuroblasoma. However, resveratrol is rapidly metabolized, mainly to its glucuronidated and sulfated derivatives. This study demonstrates that resveratrol alone, and not the glucuronidated or sulfated metabolites, is taken up into tumor cells, induces a rise in [Ca(2+)](i), and ultimately leads to a decrease in tumor cell viability. A new water-soluble resveratrol formulation was delivered directly at the site of the tumor in a neuroblastoma mouse model. The amount of unmodified resveratrol associated with the tumor increased more than 1000-fold. The increase of unmodified resveratrol associated with the tumor resulted in tumor regression. The number of residual tumor cells that remained viable also decreased as the ratio of the metabolites relative to unmodified resveratrol declined.
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PMID:Resveratrol metabolites do not elicit early pro-apoptotic mechanisms in neuroblastoma cells. 2140 Oct 48

Current therapies for high-risk neuroblastoma (NB) necessitate the availability of several aliquots of autologous peripheral blood stem cells to reverse-associated myelosuppression. Priming with the CXCR4 inhibitor plerixafor plus G-CSF was associated with successful stem cell harvest in 5/7 heavily prior-treated patients with stage 4 NB who had previously failed G-CSF priming. Minimal residual disease was not detected in harvested cells from any patient despite the presence of disease in bone/bone marrow in 6/7. Hematopoietic reconstitution was achieved in all three patients receiving plerixafor-primed stem cells after myeloablative therapy. Plerixafor is an effective and safe agent for stem cell collection in patients with NB.
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PMID:Plerixafor plus granulocyte-colony stimulating factor for autologous hematopoietic stem cell mobilization in patients with metastatic neuroblastoma. 2141 84


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