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

An 8-year-old boy with neuroblastoma of the right adrenal gland is reported. His initial treatment included chemotherapy and surgery, with complete response (CR) being achieved at the initial site. A metastatic lesion was found in the right maxillary sinus 32 months after his initial treatment. A mass in the right soft palate was detected and was clinically suspected of being a metastasis. The results of biopsy were negative and the differential diagnosis from the imaging studies of CT included odontogenic disease, fungal infection, paranasal sinus cyst or hematoma, and benign tumors. Open transantral biopsy was done under general anesthesia, revealing severe inflammation in the right maxillary sinus as well as bone erosion. The histopathological diagnosis was metastatic neuroblastoma from the adrenal lesion. The local field was irradiated with 20 Gy of linear accelerator (linac) radiation, then the local field was eradicated. Extensive skeletal metastases were subsequently found by bone scintigraphy. Despite further treatment his general condition deteriorated rapidly and he died 24 months after starting treatment. We review the previous reports and discuss metastasis to the sinuses.
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PMID:A case of infantile neuroblastoma with intramucosal metastasis in a paranasal sinus. 1099 38

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neoplasm originating from neuroepithelial cells. Treatment for this tumor has traditionally required a craniofacial resection through an external approach. The safety and efficacy of transnasal endoscopic resection is uknown. A series of five patients with Kadish stage A or B lesions of the anterior skull base were resected and immediately reconstructed endoscopically over a nine-year period. There were 2 males and 3 females with a mean age of 64 years. Three patients were treated primarily and two were treated secondarily for recurrences after failing a traditional external craniofacial resection and postoperative radiotherapy. All primary lesions received adjuvant radiotherapy. The mean follow-up time was 31 months. All patients were discharged within 4 days or less. Operative complications included temporary postoperative infraorbital anesthesia (1 patient), unplanned cerebrospinal fluid leak (1 patient), and can orbital hematoma (1 patient). None of these complications resulted in any permanent sequelae. Two patients had prolonged nasal crusting for up to one year due to a variety of factors; large cavities, radiation therapy, and exposed lyophilized dural graft. However, all skull base defects healed without any short- or long-term sequelae. There have been no local recurrences. Two patients (1 primary and 1 secondary) developed regional metastasis to the orbit or cervical lymph nodes managed with primary transorbital excision or modified neck dissection, respectively. All but one patient remain free of disease by clinical, endoscopic, and radiographic (CT or MRI) surveillance. The remaining patient recurred distally as an undifferentiated carcinoma in his temporal bone and lungs 12 months after the initial resection. In experienced hands select cases of esthesioneuroblastoma can be safely excised and reconstructed endoscopically with comparable degrees of tissue removal as with external approaches. Short-term oncologic results in this mall series of patients appear to be comparable to traditional methods. Longer follow-up on a larger series of patients is warranted.
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PMID:Endoscopic resection of esthesioneuroblastoma. 1155 61

A 16-year-old girl had suffered from chronic graft versus host disease (GVHD) caused by peripheral blood stem cell transplantation (PBSCT) after chemotherapy for neuroblastoma and pulmonary aspergillosis of the right upper lobe. She presented with hematemesis and underwent upper gastrointestinal endoscopy under general anesthesia. At the end of the examination, massive pulmonary hemorrhage occurred suddenly. A double lumen endobronchial tube was inserted for unilateral ventilation in order to control hemorrhage, and right pulmonary hemorrhage was found. Pulmonary scintigram and angiography could not demonstrate the bleeding site, and we suspected that pulmonary hemorrhage had been caused by pulmonary aspergillosis because aspergillus is known to have pathologically invasive character to the adjacent tissue and blood vessels. Despite right pneumonectomy was performed to control pulmonary hemorrhage, she died five days later from multiple organ failure. This case suggests that immediate unilateral ventilation is useful for the isolation of the bleeding lung when pulmonary hemorrhage is massive and we should know the risk of pulmonary hemorrhage in patients with pulmonary aspergillosis.
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PMID:[Intraoperative massive pulmonary hemorrhage due to pulmonary aspergillosis]. 1192 93

Increased catecholamine secretion from neuroblastomas can occasionally be demonstrated, but severe hypertension is uncommon. We report the perioperative management of a 5 year old child with stage III adrenal neuroblastoma who presented with malignant hypertension and high norepinephrine and dopamine levels. Hypertensive crises occurred during anesthesia for surgical biopsy and during chemotherapy. After blood pressure control using phenoxybenzamine and enalapril, doxazosin was used successfully as the preoperative alpha-adrenergic receptor antagonist for surgical tumor resection.
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PMID:Perioperative management of a child with severe hypertension from a catecholamine secreting neuroblastoma. 1596 Jun 47

A 3-year-old girl with neuroblastoma was scheduled for intraoperative radiation therapy. Prior to surgery, meetings were held to ensure the safety during transfer between the operating room and the radiation unit because those units were placed on the different floors in our hospital. In the operating room the patient was anesthetized with propofol, fentanyl and ketamine (PFK). After the resection of the tumor, the patient was moved to the radiation unit. All personnel had to leave the treatment room while the radiation was administered, which required 3 minutes. During the transfer and treatment, the following apparatus were effective to ensure the patient's safety; mobile respiratory and cardiovascular monitors, closed-circuit television to observe the monitors and patient, and a portable gas powered ventilator. The total intravenous anesthesia with PFK was used throughout all scheduled procedures. For patients treated with intraoperative radiation therapy, the administration of inhalation anesthetics should be discontinued to supply 100% oxygen, which enhances the effect of irradiation, and to avoid the complexity of the transfer with an anesthetic machine. This discontinuation, however, may cause the cardiovascular instability. It seems that total intravenous anesthesia has advantages over inhalation anesthesia for patients who undergo intraoperative radiation therapy.
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PMID:[Safe management of anesthesia with total intravenous anesthesia using propofol, fentanyl and ketamine for a patient treated with intraoperative radiotherapy]. 1671 19

The mu-opioid receptor (MOR1) is a target of endogenous and exogenous opioids and plays a pivotal role for anesthesia and analgesia. Variations in the 5' flanking sequence of the mu-opioid receptor gene may influence transcriptional regulation and ultimately alter protein expression of MOR1. In the present study we investigated the influence of eight single nucleotide polymorphisms (SNP) within the mu-opioid receptor promoter on promoter activity and evaluated the frequencies of the relevant SNPs in 700 patients under opioid medication. Reporter-gene-constructs were created by means of PCR and site directed mutagenesis, testing eight SNPs previously described. The neuroblastoma cell line SHSY5Y was used for transfection and promoter activity was estimated by luciferase activity. Of the eight reporter gene constructs employed to test genomic variations, two produced a significant change in luciferase activity when compared to wild-type constructs. The G-554A variation located within a known NFkB binding element resulted in a decreased activity whereas the A/G base exchange at position -1320 showed an increased luciferase activity. This particular variant generated a myeloid zinc finger (MZF1) cis-acting element known to impact transcription. The allele frequency of the -1320G variant was 0.21% in 700 Caucasian patients under opioid medication in contrast to 9.1% reported previously in drug addicted African Americans. Because of this unexpected low frequency an association analysis to opioid requirements and effects of mu-opioid receptor agonists was not feasible. In conclusion, transcriptional regulation of MOR1 is modified by two genetic variations at positions -554 and -1320 of the mu-opioid receptor promoter. Individuals presenting these variations may have an altered level of MOR expression. A possible association of these genomic variants on efficacy and side effects of opioid treatment in different ethnic groups has to be elucidated.
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PMID:Genomic variations and transcriptional regulation of the human mu-opioid receptor gene. 1684 22

We report the anesthetic management in two infants who were a three-month-old boy (case 1) and a two- month-old girl (case 2), undergoing hemilaminectomy for giant mediastinal neuroblastoma. Neuroblastoma invades into the intrathecal space in a rare occasion, resulting in paraplegia, which requires emergent operation to save the spinal cord. Mediastinal neuroblastoma also possesses significant respiratory and cardiovascular risks due to the close anatomic relationship with vital organs. In the present report, we show that preoperative computed tomography (CT) scan and/or echocardiography revealed tracheal deviation in case 1 and mass compression of the left atrium in case 2. Therefore, the patients with mediastinal masses should be carefully evaluated before subjecting them to anesthesia. Especially, there is a possibility that the masses which do not appear to compress the airway and cardiovascular systems develop the airway obstruction and cardiovascular collapse after induction of general anesthesia. In fact, before induction of general anesthesia in the case 2, trial of the prone positioning which was scheduled for the hemilaminectomy, resulted in bradycardia and hypotension, presumably due to the compression of the left atrium. In summary, the authors here discussed the preoperative evaluation and anesthetic management for giant mediastinal neuroblastoma in infants.
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PMID:[Anesthetic management in two infants undergoing hemilaminectomy for giant mediastinal neuroblastoma]. 1731 29

Bupivacaine is one of the amide type local anesthetics and is widely used for epidural anesthesia and blockade of nerves. Bupivacaine administration locally could result in neuron injury showing transient neurologic symptoms. Dexamethasone is a synthetic glucocorticoid and may exert cytoprotective properties against damage induced by some stimuli. In the present study, we evaluated the effects of dexamethasone on bupivacaine-induced toxicity in mouse neuroblastoma N2a cells. N2a cells were exposed to bupivacaine in the presence or absence of dexamethasone. After treatment, the cell viability, nuclear condensation, and lactate dehydrogenase levels were evaluated. Mitochondrial potential and Akt (threonine-serine protein kinase B) activation were also examined. In a separate experiment, we examined the effect of Akt inhibition by triciribine on cell viability following dexamethasone treatment. We also investigated whether dexamethasone could prevent lidocaine-induced neurotoxicity. Treatment of N2a cells with bupivacaine resulted in significant cell injury as evidenced by morphological changes, LDH leakage, and nuclear condensation. Pretreatment of the cells with dexamethasone significantly attenuated bupivacaine- and lidocaine-induced cell injury. Dexamethasone treatment prevented the decline of mitochondrial potential caused by bupivacaine and increased the levels of Akt phosphorylation. Importantly, pharmacological inhibition of Akt abolished the protective effect of dexamethasone against bupivacaine-induced cell injury. Our data suggest that pretreatment of neuroblastoma cells with dexamethasone exerts a protective effect on bupivacaine-induced neuronal cell injury. The mechanisms involve activating the Akt signaling pathway.
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PMID:Dexamethasone attenuated bupivacaine-induced neuron injury in vitro through a threonine-serine protein kinase B-dependent mechanism. 2003 43

The aim of this study was to evaluate the effect of diphenidol on blocking Na(+) currents and spinal anesthesia. We used the patch-clamp method to examine if diphenidol blocked Na(+) currents. Lidocaine, a common used local anesthesia, was used as control. We also evaluated the potencies and durations of diphenidol and lidocaine on spinal blockades of motor function, proprioception, and nociception in rats. Lidocaine exhibited a concentration- and state-dependent effect on tonic blockade of voltage-gated Na(+) currents in mouse neuroblastoma N2A cells (IC(50) of 8.1 and 138.9 microM at holding potentials of -70 and -100 mV, respectively). Diphenidol was more potent (IC(50) of 0.77 and 62.6 microM at holding potentials of -70 and -100 mV, respectively). However, unlike lidocaine, block of Na(+) currents by diphenidol lacked use-dependence. We also found that diphenidol acted like lidocaine and produced dose-related spinal blockades of motor function, proprioception and nociception. Although diphenidol had similar potencies of spinal anesthesia compared with lidocaine it produced a much longer duration of spinal blockades than lidocaine. Our results demonstrated that intrathecal diphenidol produced a long duration and similar potency on spinal anesthesia compared with lidocaine in rats. The anesthetic effect of diphenidol could be in part due to its blockade of Na(+) currents.
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PMID:Diphenidol inhibited sodium currents and produced spinal anesthesia. 2017 39

The purpose was to quantify the setup margin for pediatric patients with neuro-blastoma using cone beam CT imaging (CBCT) and ultrasound localization. Ten patients, with a median age of 4.3 years (1.8 to 7.9) underwent daily pretreatment localization CBCT and every other day post-treatment CBCT to calculate interfractional and intrafraction movement. Localization was based on CBCT to treatment planning CT registration in the lumbar spine region. Each subject was treated in the supine position under IV general anesthesia using intensity-modulated radiation therapy. Patients were repositioned based on the daily pretreatment CBCT. Required setup margins based on inter- and intrafraction positioning errors were calculated based on weekly and daily imaging scenarios. Four patients had ultra-sound localization of the kidneys performed before the CBCT. Correlation between daily CBCT and ultrasound was investigated. A lateral, longitudinal and vertical setup margin of 5.4, 5.6, and 5.9 mm is required without daily CBCT. When daily CBCT was incorporated, the setup margin was reduced to 1.5, 2.1, and 1.7 mm. There was no correlation between the suggested ultrasound shifts and the shifts based on the CBCT. Daily localization based on CBCT of the lumbar spine can reduce the required setup margin for neuroblastoma patients, thereby reducing normal tissue exposure for this young patient population. The internal margin needs further investigation before PTV reduction can be made. Ultrasound localization was highly variable and not correlated to CBCT shifts.
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PMID:Daily image-guided localization for neuroblastoma. 2108 96


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