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)

We present the case of a 3-year-old child with neuroblastoma metastatic to the bone marrow. [123I]MIBG total body scintigraphy revealed a diffuse pattern of intense fixation throughout the skeleton. This was analogous to the "super scan" described with 99mTc-MDP bone scintigraphy when extensive metastatic or metabolic bone disease is present. Essentially, a high bone-to-soft tissue ratio was found without uptake in the liver, kidney and bladder, owing to a "steal" mechanism caused by the avid bone metastases.
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PMID:Diffuse bone marrow uptake [123I]MIBG in neuroblastoma: an "MIBG super scan" case report. 857 5

Localization of skeletal tracer in a neuroblastoma primary is common but localization in extraskeletal metastatic sites has not received recognition. Tc-99m MDP concentration in hepatic or pulmonary metastases was noted in three of ten patients with such metastases. Lesion size appears to be important for demonstrating these metastases with Tc-99m MDP. This was particularly true for hepatic metastases, which were identified only when they were 5 cm or greater in diameter.
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PMID:Localization of Tc-99m MDP in neuroblastoma metastases to the liver and lung. 885 16

In a group of 97 patients aged from 6 months to 12 years, all with suspected or proven neural crest tumours, metaiodobenzylguanidine (MIBG) scintigraphy was performed at the time of diagnosis and, in some instances, after induction chemotherapy. All the patients underwent a tumour biopsy with cytological and histological analysis, in addition to imaging examinations such as X-rays, ultrasound, computed tomography and magnetic resonance, within a short period before or after scintigraphy. In 82 of 97 cases MIBG was effective in detecting the primary tumour, hence the technique's sensitivity was 84%. A significant different of sensitivity between [131I]MIBG and [123I]MIBG was not demonstrated. As regards metastatic locations, MIBG scans revealed one or more bone metastases in 12 cases, bone marrow involvement (assumed to be present when diffuse and symmetric uptake in the spine, pelvis and possibly other skeletal sites were visualized) in 9 cases, and focal liver metastases or hepatomegaly in 4 cases. Probably owing to the restrictive diagnostic criterion adopted or to the early phase of the bone marrow involvement, the last was found by biopsy but missed by MIBG in 25 cases. The overall sensitivity in detecting metastases was low (48%), but it was much higher if only bone metastases were considered (81%). Twenty-nine patients who had positive scans at diagnosis were checked following 1-2 courses of induction chemotherapy (IC). MIBG scans remained positive in 22 primary tumours, while 7 primary masses were no longer detected. Out of 12 cases showing metastases at diagnosis, two cases with liver lesions became normal and in one case some, but not all, of the bone lesions were not detectable; 4 cases remained abnormal, while in 5 cases bone marrow involvement was not confirmed. Three cases were confirmed to be true negatives; in 4 other cases bone marrow involved not showing at diagnosis was revealed and confirmed by biopsy; 3 cases in which bone marrow involvement was not revealed by MIBG at diagnosis, had normal MIBG and biopsy results after IC; finally, 2 false negative bone marrow cases and 5 true negative cases at diagnosis remained unchanged, but were not checked by biopsy. Performing total body MIBG scintigraphy in childhood neuroblastoma at diagnosis is useful: 1) to predict the nature of the masses detected by other imaging techniques, when biopsy has not yet been performed; 2) for more accurate tumour staging, in addition to standard imaging investigations, MDP scintigraphy and bone marrow aspiration biopsy, thanks to its ability to detect metastatic lesions; 3) to anticipate the decrease in sensitivity of the technique in detecting both the primary mass and the metastases following induction chemotherapy.
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PMID:Radioiodinated meta-iodobenzylguanidine in the diagnosis of childhood neuroblastoma. 900 44

Tc-99m sestamibi, originally developed for myocardial studies, has been used as a tumor-seeking agent. Recently, the agent also was reported to be a functional tracer to predict multidrug resistance-related p-glycoprotein expression in tumor tissue. The current report presents the authors' experience with sestamibi tumor scintigraphy in a neuroblastoma. Although I-131 MIBG tumor imaging and Tc-99m MDP bone scanning accurately demonstrated the extent of the disease, Tc-99m sestamibi showed no accumulation in primary and metastatic foci. Lack of sestamibi uptake was initially thought to be suggestive of failure to respond to chemotherapy because of p-glycoprotein expression. However, the patient responded well to chemotherapy and complete remission was achieved. The failure of Tc-99m sestamibi to detect a neuroblastoma and the lack of sestamibi accumulation in the tumor may not always be related to chemotherapy resistance.
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PMID:False-negative scintigraphy with Tc-99m sestamibi in stage IV neuroblastoma. 1046 25

A case of a 30 year old male with an eight year history of neuroblastoma and whose general health was good is presented. After his last check-up, which included a CT scan and 99mTc-MDP bone scintigraphy, a 123I-MIBG and 111In-DTPA-D-Pheoctreotide scintigraphy was performed and provided us with complementary data that contributed to a more precise diagnosis of the location and extension of the neuroblastoma and to the biological features of the tumor. Thus, this report deals with an adult neuroblastoma patient whose general health is good in whom the exact extension of the lesion was determined by a combination of diagnostic imaging techniques.
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PMID:[Eight year history of neuroblastoma in an adult patient. Value of 123I-MIBG and 111In-DTPA-D-Phe-octreotide scintigraphy]. 1048 Nov 14

In this study, we investigated prospectively the diagnostic role of 99Tcm-MIBI for staging and for predicting the therapeutic response of stage IV neuroblastoma compared with 131I-MIBG imaging and 99Tcm-MDP bone scintigraphy. Nine patients (4 girls and 5 boys aged 1-7 years) with suspected or proven stage IV neuroblastoma were studied with 99Tcm-MIBI at initial diagnosis and after 12-18 months of multidrug therapy. After the injection of 80 MBq.kg-1 99Tcm-MIBI, early (10 min) and delayed (1 h) images were obtained. The data were correlated with 131I-MIBG scans, bone scintigraphy, ultrasound, computed tomography and/or magnetic resonance imaging, and bone marrow biopsy. Eight of nine primary tumours and 41 metastatic lesions were detected by 131I-MIBG scintigraphy. None of the primary lesions demonstrated significant 99Tcm-MIBI accumulation. Sestamibi was positive in 16 of 41 MIBG-avid metastatic lesions. After six courses of multidrug chemotherapy, 30 131I-MIBI-avid neuroblastoma metastases that were 99Tcm-MIBI-negative at the time of diagnosis still did not show significant sestamibi accumulation. Follow-up demonstrated that all lesions that were 99Tcm-MIBI-avid at the time of diagnosis remained negative. Of these 16 lesions, seven were positive for 131I-MIBG accumulation with no reduction in size, and nine showed resolution after therapy. New metastatic foci detected by MIBG scintigraphy did not accumulate 99Tcm-MIBI. Clinical evaluation of patients with no 99Tcm-MIBI uptake in primary and secondary sites of neuroblastoma confirmed that they were resistant to multidrug chemotherapy. All 99Tcm-MIBI-positive lesions, irrespective of clinical outcome, demonstrated significant clearance of tracer on the delayed images. We conclude that 99Tcm-MIBI has no role in the staging of neuroblastoma. Sestamibi is a well-documented transport substrate for P-glycoprotein-related multidrug resistance and serial imaging may provide prognostic information on the therapeutic value of chemotherapy.
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PMID:The role of 99Tcm-sestamibi scintigraphy in the staging and prediction of the therapeutic response of stage IV neuroblastoma: comparison with 131I-MIBG and 99Tcm-MDP scintigraphy. 1057 8

In vitro transcription/translation studies with model proteins have shown that glycosylation of Asn-Xaa-Thr sequons is reduced when the sequon is within 60 residues of the C-terminus of the protein. We have previously shown that in living cells N-glycosylation of the prion protein (PrP) is also abolished when its Asn-Ile-Thr and Asn-Phe-Thr sequons are less than 60 residues from the C-terminus (Walmsley and Hooper [2003] Biochemical Journal, 370, 351-355). To investigate whether sequon distance to the C-terminus is a general determinant of N-glycosylation in living cells, Asn-Ile/Phe-Thr sequons were introduced into another glycosylphosphatidylinositol (GPI) anchored protein, membrane dipeptidase (MDP), at similar distances from the C-terminus as those in PrP. When expressed in the human neuroblastoma SH-SY5Y cell line, the introduced sequons were fully N-glycosylated even when they were less than 60 residues from the C-terminus in both GPI-anchored and secreted forms of MDP. These data demonstrate that the utilization of sequons in some proteins is independent of their distance from the C-terminus.
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PMID:Glycosylation efficiency of Asn-Xaa-Thr sequons is independent of distance from the C-terminus in membrane dipeptidase. 1277 76

The hypothalamic galanin-like peptide (GALP) was isolated by its ability to activate galanin receptors. The mature porcine GALP is a 60-amino acid neuropeptide proteolytically processed from a 120-amino acid precursor protein. It contains a region identical to the N-terminal 13-amino acids of the neuropeptide galanin. Within the sequence of human GALP (1-60) a potential proteolytic cleavage site between two basic amino acids is present at position 33, which might lead to a shorter C-terminally amidated peptide. In addition, the first two amino acids could be potentially removed via the action of dipeptidase IV. Ligand binding assays using the human neuroblastoma cell line SH-SY5Y transfected with the respective galanin receptors revealed that human GALP (1-60) displayed the highest affinity for the galanin receptor subtype GalR3 (IC50 = 10 nM) followed by GalR2 (IC50 = 28 nM) and GalR1 (IC50 = 77 nM). Ligand binding assays and functional studies showed that the human GALP (3-32) fragment was at least as potent as full length GALP (1-60). Other studies have shown that shorter fragments like human GALP (1-21) and GALP (22-60) were not effective on feeding responses in mice as compared to the full length peptide. Taken together these data suggest that the putative fragment GALP (3-32) might represent the strongest mediator of biological GALP activity. Furthermore it might be a useful tool to study the affinity of GALP to galanin receptors and to search for specific GALP receptors.
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PMID:Pharmacological and functional characterization of galanin-like peptide fragments as potent galanin receptor agonists. 1594 9

A Tc MDP bone superscan occurs when osseous activity is extremely intense and genitourinary and soft tissue activity is not identified. A similar phenomenon has been described with metaiodobenzylguanidine (MIBG) in metastatic pheochromocytoma and neuroblastoma. We present a case of metastatic paraganglioma resulting in an MIBG superscan. Neuroendocrine bone metastasis alters the biodistribution of MIBG such that the liver, heart, and urinary bladder are not well visualized. Our case occurred in association with neurofibromatosis type 1 and in the absence of an identified primary tumor.
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PMID:MIBG superscan of metastatic paraganglioma occurring with neurofibromatosis type 1. 2345 26

Neuroblastoma is the third most common malignant solid tumor of childhood. It originates from primitive neural crest cells of the sympathetic nervous system. Many imaging procedures help guide therapy and predict outcomes. Anatomic imaging methods, such as CT and MRI, are most useful for evaluation of the primary tumor mass and nearby involved lymph nodes. Functional imaging tracers, such as [123I]MIBG, [18F]FDG, and [99mTc]MDP, are used to assess the extent of disease and to search for distant metastases. [123I]MIBG is the principal functional imaging tracer for the detection and monitoring of neuroblastoma. [18F]FDG PET/CT is an alternative that is valuable in tumors with poor or no MIBG-uptake. [99mTc]MDP bone scans may be useful to assess cortical bone metastases. This article will review the use of [123I]MIBG and other functional imaging agents for the management of patients with neuroblastoma.
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PMID:Functional-metabolic imaging of neuroblastoma. 2347 31


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