National Cancer Institute®
Last Modified: February 1, 2002
UI - 11591983
AU - Iwanaka T; Arai M; Ito M; Kawashima H; Yamamoto K; Hanada R; Imaizumi S
TI - Surgical treatment for abdominal neuroblastoma in the laparoscopic era.
SO - Surg Endosc 2001 Jul;15(7):751-4
AD - Departments of Surgery, Saitama Children's Medical Center, 2100 Magome, Iwatsuki, Saitama 339-8551, Japan. firstname.lastname@example.org
BACKGROUND: The role of laparoscopy in children with cancer has not been fully defined. The aims of this study were to develop an optimal surgical procedure for the treatment of abdominal neuroblastoma in the laparoscopic era and to evaluate the advantages and disadvantages of children were diagnosed with abdominal neuroblastoma at our center, and 44 surgical procedures were performed on them. Patients with advanced neuroblastoma underwent laparoscopic biopsy, open biopsy, and delayed primary or second-look excision, whereas early neuroblastoma cases had either laparoscopic or open excision. We compared the length of the operation, intraoperative blood loss, length of hospital stay, complications, and time to start postoperative feeding and chemotherapy for the laparoscopic and open surgery groups. RESULTS: Length of stay and time to postoperative feeding and chemotherapy were significantly lower in the laparoscopic group than the open surgery group. However, there were no significant differences between the two groups in length of operation and intraoperative blood loss. CONCLUSION: Laparoscopic biopsy and excision of abdominal neuroblastoma are effective and efficient surgical procedures in children.
UI - 11690566
AU - Barber DS; Ehrich M
TI - Esterase inhibition in SH-SY5Y human neuroblastoma cells following exposure to organophosphorus compounds for 28 days.
SO - In Vitr Mol Toxicol 2001 Summer;14(2):129-35
AD - Virginia-Maryland Regional College of Veterinary Medicine, Blacksburg, Virginia 24061, USA.
Esterase inhibition was determined in SH-SY5Y human neuroblastoma cells grown in serum-free media and exposed to 10(-11) to 10(-7) M concentrations of organophosphorus (OP) compounds for 28 days. To examine metabolic activation in these exposures, pairs of pro- and active toxicants were studied, including chlorpyrifos and its oxon, parathion and paraoxon, and tri-ortho-tolyl phosphate and phenyl saligenin phospahte. Inhibition of acetylcholinesterase was greater in cells treated for 28 days with all active organophosphorus compounds than it was in cells treated only once with the same concentration of a given OP compound. The protoxicants chlorpyrifos and parathion produced acetylcholinesterase inhibition after multiple exposures although no inhibition was seen following a single exposure to these agents. Exacerbation of neurotoxic esterase inhibition by multiple exposures to the test compounds was not as pronounced as that of acetylcholinesterase. Exposure to the test compounds for 28 days did not significantly enhance esterase inhibition produced by a subsequent exposure to 10(-9) M chlorpyrifos-oxon. The results indicate that in vitro methods can be used to study the effect of multiple OP exposures on esterase activity.
UI - 11736646
AU - Jori FP; Galderisi U; Piegari E; Peluso G; Cipollaro M; Cascino A;
TI - Giordano A; Melone MA RB2/p130 ectopic gene expression in neuroblastoma stem cells: evidence of cell-fate restriction and induction of differentiation.
SO - Biochem J 2001 Dec 15;360(Pt 3):569-77
AD - Department of Neurological Sciences, Second University of Naples, Via Pansini 5, 80100 Naples, Italy.
The activity of the RB2/p130 gene, which is a member of the retinoblastoma gene family, is cell-cycle-regulated and plays a key role in growth inhibition and differentiation. We used neuroblastoma cell lines as a model for studies on neural crest progenitor cell differentiation. We show that Rb2/p130 ectopic protein expression induces morphological and molecular modifications, promoting differentiation of intermediate (I) phenotype SK-N-BE(2)-C neuroblastoma cells towards a neuroblastic (N) rather than a Schwann/glial/melanocytic (S) phenotype. These modifications are stable as they persist even after treatment with an S-phenotype inducer. Rb2/p130 ectopic expression also induces a more differentiated phenotype in N-type SH-SY-5Y cells. Further, this function appears to be independent of cell-cycle withdrawal. The data reported suggest that the Rb2/p130 protein is able to induce neuronal lineage specification and differentiation in neural crest stem and committed neuroblastoma cells, respectively. Thus, the Rb2/p130 protein seems to be required throughout the full neural maturation process.
UI - 11815985
AU - Fakhari M; Pullirsch D; Abraham D; Paya K; Hofbauer R; Holzfeind P;
TI - Hofmann M; Aharinejad S Selective upregulation of vascular endothelial growth factor receptors neuropilin-1 and -2 in human neuroblastoma.
SO - Cancer 2002 Jan 1;94(1):258-63
AD - Department of Pediatric Surgery, University of Vienna, Vienna, Austria.
BACKGROUND: Recent studies show that vascular endothelial growth factor (VEGF) and its receptors Flt-1 and KDR, and a series of other angiogenic molecules, are upregulated in advanced but not low stage human neuroblastoma. Neuropilin-1 and 2 (NRP) are novel specific receptors of VEGF(165), whose role is unknown in human neuroblastoma. METHODS: Tissue biopsies of 37 children with Stage I-IV neuroblastoma were obtained, as well as biopsies of 7 normal adrenals as controls. The mRNA expression of VEGF(165) and its receptors Flt-1, KDR, NRP1, and NRP2 was evaluated by real-time reverse transcription polymerase chain reaction. NRP protein expression was detected by immunocytochemistry and Western blotting. RESULTS: VEGF(165) mRNA was upregulated in Stage III and IV and Flt-1 and KDR gene expression was increased in Stage III, while NRP1 and 2 mRNA and protein levels were higher in Stages I-IV vs. controls (P < 0.05). NRP was expressed in vascular endothelial but not tumor cells. CONCLUSIONS: These results show for the first time that human neuroblastoma expresses NRP, and that NRP co-regulates VEGF angiogenic effect in human neuroblastoma. NRP might be a sensitive angiogenic measure of VEGF systems in neuroblastoma, particularly in its early stages. Copyright 2002 American Cancer Society.
UI - 11807803
AU - Dennis SL; Manji SS; Carrington DP; Scarcella DL; Ashley DM; Smith PJ;
TI - Algar EM Expression and mutation analysis of the Wilms' tumor 1 gene in human neural tumors.
SO - Int J Cancer 2002 Feb 10;97(5):713-5
AD - Murdoch Children's Research Institute, Parkville, Victoria, Australia.
The Wilms' tumor 1 gene, WT1, encodes a zinc-finger protein that is implicated in the development of Wilms' tumor. Mutant or aberrantly expressed WT1 isoforms have also been described in desmoplastic small round cell tumor, acute leukemias, mesothelioma, breast tumors and melanoma. During early development, WT1 is expressed in the brain and spinal cord, however its role in the malignancies that affect these tissues has not been previously investigated. In our study we have examined neural tumors including brain tumors and neuroblastomas for WT1 expression and for mutations affecting the zinc-fingers. Although WT1 expression was detected in gliomas, medulloblastomas and neuroblastomas, neither zinc-finger region mutations nor splicing anomalies affecting the KTS site were detected. We therefore conclude that WT1 does not play a significant role in the etiology of human neural tumors. Copyright 2001 Wiley-Liss, Inc.
UI - 10918246
AU - Biasotti S; Garaventa A; Villavecchia GP; Cabria M; Nantron M; De
TI - Bernardi B False-negative metaiodobenzylguanidine scintigraphy at diagnosis of neuroblastoma.
SO - Med Pediatr Oncol 2000 Aug;35(2):153-5
AD - Department of Pediatric Hematology-Oncology, Giannina Gaslini Institute, Genova, Italy.
UI - 11496360
AU - Chan GC; Leung YL; Shing MM; Luk CW; Ling SC; Lee AC
TI - Does a "false negative" MIBG scan predict a better outcome in neuroblastoma patients?
SO - Med Pediatr Oncol 2001 Aug;37(2):155
UI - 11743648
AU - Cinatl J Jr; Kotchetkov R; Blaheta R; Driever PH; Vogel JU; Cinatl J
TI - Induction of differentiation and suppression of malignant phenotype of human neuroblastoma BE(2)-C cells by valproic acid: enhancement by combination with interferon-alpha.
SO - Int J Oncol 2002 Jan;20(1):97-106
AD - Institute for Medical Virology, Johann Wolfgang Goethe-University Medical Center, D-60596 Frankfurt am Main, Germany. email@example.com
Valproic acid (VPA) has been shown to induce growth-arrest and differentiation of human neuroectodermal tumors similarly to several other fatty acids. In the present study, we show that continuous VPA treatment together with Interferon-alpha (INF-alpha) synergistically inhibited cell growth of a well-established model of neuroblastoma (NB) differentiation using the human N-myc amplified cell line BE(2)-C. Suppression of tumor growth was accompanied by morphological features of neuronal differentiation and inhibition of histone deacetylase activity. Furthermore, induction of differentiation was concomitant with altered expression of genes related to malignant phenotype such as down-regulation of N-myc, induction of bcl-2 and neural cell adhesion molecule. Production of inhibitors of angiogenesis like thrombospondin-1 and activin A was up-regulated in differentiated NB cells. Treatment with VPA alone decreased the ability of BE(2)-C cells to adhere to and penetrate human endothelium. All these effects of VPA were significantly enhanced when combined with INF-alpha which on its own had little or no effect. These results suggest that combination of VPA and INF-alpha may provide a novel therapeutic strategy for NB due to enhanced inhibition of tumor cell growth, induction of tumor differentiation and suppression of malignant biology by reduced angiogenic and decreased metastatic potentials.
UI - 11781663
AU - Narita M; Bahar R; Hatano M; Kang MM; Tokuhisa T; Goto S; Saisho H;
TI - Sakiyama S; Tagawa M Tissue-specific expression of a suicide gene for selective killing of neuroblastoma cells using a promoter region of the NCX gene.
SO - Cancer Gene Ther 2001 Dec;8(12):997-1002
AD - Division of Pathology, Chiba Cancer Center Research Institute, Chiba, Japan.
The human NCX gene, a homologue of the murine neural crest homeobox (Ncx/Hox11L.1) gene whose expression is restricted to a subset of neural crest-derived tissues, was expressed in human neuroblastoma cells but not in other tumors or fibroblasts. A 4.5-kb genomic fragment in the 5'-flanking region of the NCX gene efficiently transcribed the fused luciferase reporter gene in human neuroblastoma cells but not in non-neuroblastoma cells. Sequential deletion of this regulatory region from the 5' side demonstrated that a 1.7-kb fragment upstream from the start codon retained the preferential promoter activity in neuroblastoma cells. The transcriptional activation by the NCX promoter was stronger than that by the SV40 T antigen promoter in human neuroblastoma cells. Transfection of neuroblastoma cells with the NCX promoter-linked herpes simplex virus-thymidine kinase (HSV-TK) gene increased their sensitivity to ganciclovir. The regulatory region of the NCX gene is thus useful for neuroblastoma-specific suicide gene therapy.
UI - 11788059
AU - Woollacott AJ; Simpson PB
TI - High throughput fluorescence assays for the measurement of mitochondrial activity in intact human neuroblastoma cells.
SO - J Biomol Screen 2001 Dec;6(6):413-20
AD - Department of Biochemistry, Merck Sharp and Dohme Research Laboratories, Neuroscience Research Centre, Harlow, Essex, UK.
The mitochondrial permeability transition event is implicated in the activation phase of apoptosis and necrosis, and is therefore postulated to play a role in many disease states. Mitochondrial permeability transition is therefore of increasing pharmaceutical interest. Drug discovery requires the rapid screening of compound libraries to identify functionally active ligands. We report the development of two fluorescence-based approaches for screening compound libraries for effects on mitochondrial function. These assays use the fluorometric imaging plate reader in 96-well format, and two commercially available dyes: JC-1 and calcein-AM. We show here that a JC-1 assay proved highly amenable to HTS implementation. By combining this with a calcein-based assay, these approaches gave complementary information: JC-1 facilitates the discovery of modulators of mitochondrial polarization from a library of approximately 100,000 compounds screened at 8 microM, and the calcein assay identifies permeability transition pore-specific inhibitors.
UI - 11720446
AU - Peaston AE; Gardaneh M; Franco AV; Hocker JE; Murphy KM; Farnsworth ML;
TI - Catchpoole DR; Haber M; Norris MD; Lock RB; Marshall GM MRP1 gene expression level regulates the death and differentiation response of neuroblastoma cells.
SO - Br J Cancer 2001 Nov 16;85(10):1564-71
AD - Children's Cancer Institute Australia for Medical Research, PO Box 81, Randwick, NSW 2031, Australia.
We have previously reported a strong correlation between poor prognosis in childhood neuroblastoma (NB) patients and high-level expression of the transmembrane efflux pump, Multidrug Resistance-associated Protein (MRP1), in NB tumour tissue. In this study, we inhibited the endogenous expression of MRP1 in 2 different NB tumour cell lines by stably transfecting an MRP1 antisense expression vector (MRP-AS). Compared with control cells, MRP-AS transfectant cells demonstrated a higher proportion of dead and morphologically apoptotic cells, spontaneous neuritogenesis, and, increased synaptophysin and neurofilament expression. Bcl-2 protein expression was markedly reduced in MRP-AS cells compared to controls. Conversely, we found that the same NB tumour cell line overexpressing the full-length MRP1 cDNA in sense orientation (MRP-S) demonstrated resistance to the neuritogenic effect of the differentiating agent, all-trans-retinoic acid. Taken together, the results suggest that the level of MRP1 expression in NB tumour cells may influence the capacity of NB cells for spontaneous regression in vivo through cell differentiation and death.
UI - 11724258
AU - Prado GL; Itabashi Y; Noda H; Miura H; Mariya Y; Abe Y
TI - Olfactory neuroblastoma visualized by Technetium-99m-ECD SPECT.
SO - Radiat Med 2001 Sep-Oct;19(5):267-70
AD - Department of Radiology, Hirosaki University School of Medicine, Japan.
We describe a case of olfactory neuroblastoma diagnosed by 99mTc-ECD SPECT. Although MRI and CT are very important for delineating these tumors, they are, by no means, specific for neuroblastomas. 131I-MIBG scintigraphy, the standard method for imaging tumors of neural crest origin, also failed to detect a histologically proven esthesioneuroblastoma.
UI - 11813175
AU - Kerbl R; Ambros IM; Ambros PF; Lackner H; Dornbusch HJ; Urban CE
TI - Neuroblastoma with focal MYCN amplification and bone marrow infiltration: a staging and treatment dilemma.
SO - Med Pediatr Oncol 2002 Feb;38(2):109-11
AD - Department of Pediatrics, Division of Hematology and Oncology, University of Graz, Graz, Austria. firstname.lastname@example.org
UI - 11813176
AU - Brodeur GM
TI - Significance of intratumoral genetic heterogeneity in neuroblastomas.
SO - Med Pediatr Oncol 2002 Feb;38(2):112-3
AD - Division of Oncology, The Children's Hospital of Philadelphia, Abramson Research Center, Philadelphia, Pennsylvania 19104-4318, USA. email@example.com
UI - 11705866
AU - Omura-Minamisawa M; Diccianni MB; Chang RC; Batova A; Bridgeman LJ;
TI - Schiff J; Cohn SL; London WB; Yu AL p16/p14(ARF) cell cycle regulatory pathways in primary neuroblastoma: p16 expression is associated with advanced stage disease.
SO - Clin Cancer Res 2001 Nov;7(11):3481-90
AD - Department of Pediatrics/Hematology-Oncology, University of California San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103, USA.
p16 regulates the G(1)-S cell cycle transition by inhibiting the cyclin D-cyclin-dependent kinase (CDK)4/CDK6-mediated phosphorylation of retinoblastoma protein (pRb). We examined the possible derangement of the p16-CDK/cyclin D-pRb pathway in 40 primary neuroblastomas including 18 samples in the unfavorable stages (C and D) and 22 in the favorable stages (A, B, and Ds) by PCR, reverse transcription-PCR, Western blot, and immunohistochemistry and correlated the results with clinical outcome. No samples harbored alterations of the p16 gene. Interestingly, the samples in the unfavorable stages exhibited expression of p16 mRNA and protein more frequently than those in the favorable stages [mRNA, 9 of 18 (50%) versus 2 of 22 (9%), P = 0.006; protein, 5 of 16 (31%) versus 0 of 18 (0%), P = 0.013]. Alterations of the downstream components of the pathway were infrequent. pRb was deregulated in the majority of samples investigated [27 of 33 (82%), 24 with hyperphosphorylated pRb and 3 with no pRb protein]. The phosphorylation status of pRb did not correlate with p16 protein expression, suggesting that the elevated p16 protein may not be functioning properly to regulate the pathway. Among patients of all stages, p16 expression was significantly associated with a lower overall survival. There was no overexpression of MDM2, and loss of p14(ARF) expression and p53 mutation were infrequent events. Taken together, these findings suggest that up-regulated p16 expression may represent a unique feature of aggressive neuroblastoma.
UI - 11843557
AU - Chung J; Park ST; Jang J
TI - Fine needle aspiration cytology of metastatic olfactory neuroblastoma: a case report.
SO - Acta Cytol 2002 Jan-Feb;46(1):40-5
AD - Department of Diagnostic Pathology, Asan Kangnung Hospital, KangNungSi, South Korea. firstname.lastname@example.org
BACKGROUND: Olfactory neuroblastoma (ONB) is an uncommon tumor, presenting as a polypoid mass arising from the upper nasal cavity. This tumor has been seldom diagnosed by direct fine needle aspiration (FNA). CASE: Metastatic ONB was diagnosed by FNA. The patient was a 40-year-old female with a polypoid mass in the nasal cavity and ipsilateral cervical lymphadenopathy. The punch biopsy of the nasal tumor revealed a smudged small round cell neoplasm with neuroendocrine differentiation, consistent with ONB. In FNA smears from the cervical lymph node, there were well-preserved, small, monotonous cells with hyperchromatic nuclei, fibrillary cytoplasm and indistinct cell borders. Also noteworthy were occasional pseudorosettes as well as rare true rosettes. By immunocytochemistry, tumor cells were positive for cytokeratin, chromogranin and synaptophysin. CONCLUSION: ONB, like adrenal neuroblastoma, shows distinctive cytologic features, including a rosette or pseudorosette and fibrillary network. FNA can accurately demonstrate these characteristic findings, and in some cases it may be a better diagnostic modality than incisional biopsy.
UI - 11773546
AU - Mitchell WG; Davalos-Gonzalez Y; Brumm VL; Aller SK; Burger E; Turkel
TI - SB; Borchert MS; Hollar S; Padilla S Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae.
SO - Pediatrics 2002 Jan;109(1):86-98
AD - Neurology Division, Childrens Hospital Los Angeles, Los Angeles, California, USA. email@example.com
OBJECTIVE: Opsoclonus-ataxia, also called "dancing eye syndrome," is a serious neurologic condition that is often a paraneoplastic manifestation of occult neuroblastoma in early childhood. Despite resection of tumor and immunosuppressive therapy, outcome generally includes significant developmental and behavioral sequelae. There is controversy about how treatment alters outcome. The goals of this study were to understand the ongoing neurologic and developmental deficits of children who are treated for opsoclonus-ataxia with associated neuroblastoma; to relate treatment history to outcome; and to quantify objectively the acute changes in motor function, speech, mood, and behavior related to intravenous immunoglobulin (IVIg) treatment. METHODS: Patients were children with opsoclonus-ataxia caused by neuroblastoma, regardless of interval since diagnosis. Records were reviewed, and children underwent comprehensive evaluations, including neurologic examination and tests of cognitive and adaptive function, speech and language, and fine and gross motor abilities. Psychiatric interview and questionnaires were used to assess current and previous behavior. In 6 children, a videotaped standardized examination of eye movements was performed. Additional examinations were performed immediately before and 2 to 3 days after treatment with IVIg in 5 children. RESULTS: Seventeen children, ages 1.75 to 12.62 years, were examined. All had a stage I or II neuroblastoma resected 3 months to 11 years previously. None received any other treatment for the tumor. All but 1 had received at least 1 year of either oral corticosteroids or corticotropin (ACTH); 12 had received 1 or more courses of IVIg, 2 g/kg. Three had received other immunosuppressive treatment, including cyclophosphamide. Cognitive development and adaptive behavior were delayed or abnormal in nearly all children. Expressive language was more impaired than receptive language. Speech was impaired, including both intelligibility and overall output. Fine and gross motor abilities were impaired. Increased age was strikingly associated with lower scores in all areas. Behavioral problems early in the course included severe irritability and inconsolability in all; later, oppositional behavior and sleep disorders were reported. Opsoclonus abated in all, but abnormalities in pursuit eye movements were found in all 6 children cooperative with standardized examination. Outcome did not differ in children who were treated with ACTH versus oral steroids. Three children who had received cyclophosphamide fared poorly. Immediate versus delayed treatment was not associated with better outcome. IVIg improved both gross and fine motor and speech function acutely, but we could not confirm long-term benefit of IVIg. Total number of courses of IVIg was not associated with outcome. CONCLUSIONS: Opsoclonus-ataxia caused by neuroblastoma causes substantial developmental sequelae that are not adequately prevented by current treatment. The increased deficits in older children raise concern that this represents a progressive encephalopathy rather than a time-limited single insult. Although the study is cross-sectional and neither randomized nor blinded, we were unable to confirm a purported advantage of either ACTH over corticosteroids or of cyclophosphamide. A randomized study is needed but is difficult for this rare condition.
UI - 11773586
AU - Ueno S; Yokoyama S; Hirakawa H; Yabe H; Suzuki Y; Atsumi H; Matsumae M
TI - Use of real-time magnetic resonance guidance to assist bone biopsy in pediatric malignancy.
SO - Pediatrics 2002 Jan;109(1):E18
AD - Department of Pediatric Surgery, Tokai University, School of Medicine, Kanagawa, Japan. firstname.lastname@example.org
Magnetic resonance (MR) imaging (MRI) has the advantage of demonstrating lesions not visualized by other radiologic modalities. We present a case involving pediatric malignancy where MR-guided bone biopsy confirmed correct histologic diagnosis and was used to plan additional treatment. A 2-year, 9-month-old boy had a history of spontaneous regression of stage 4S neuroblastoma. (123)I-metaiodobenzylguanidine scintigraphy showed a hot spot at his right lower leg; however, neither plain radiograph (99m)Tc diphosphonate bone scan was positive. Only MRI depicted a lesion at the distal third of his right tibia, and a subsequent MR-guided bone biopsy was diagnostic of bone marrow metastasis. After 6 courses of intensive chemotherapy, he has been in complete remission. MR-guided biopsy technique is likely to be particularly useful for the resection of invisible metastatic lesions, especially those that are only visible using MRI.
UI - 11754965
AU - Kitamuro T; Takahashi K; Totsune K; Nakayama M; Murakami O; Hida W;
TI - Shirato K; Shibahara S Differential expression of adrenomedullin and its receptor component, receptor activity modifying protein (RAMP) 2 during hypoxia in cultured human neuroblastoma cells.
SO - Peptides 2001 Nov;22(11):1795-801
AD - Department of Molecular Biology and Applied Physiology, Tohoku University School of Medicine, 980-8575, Sendai, Miyagi, Japan.
Adrenomedullin is a potent vasodilator peptide originally isolated from a pheochromocytoma. Recently, a novel adrenomedullin receptor has been identified as a complex consisting of calcitonin receptor-like receptor (CRLR) and receptor activity modifying protein (RAMP) 2. To explore possible pathophysiological roles of adrenomedullin and its receptor component RAMP2 in hypoxic tissues, we studied effects of hypoxia on expression of adrenomedullin and RAMP2 in two human neuroblastoma cell lines, IMR-32 and NB69, by radioimmunoassay and Northern blot analysis. Expression levels of adrenomedullin were increased by hypoxia in both cell lines. Treatment with cobalt chloride or desferrioxamine mesylate also increased expression levels of adrenomedullin mRNA. On the other hand, expression levels of RAMP2 mRNA were decreased in IMR-32 cells and were not changed in NB69 cells by hypoxia. Treatment with cobalt chloride or desferrioxamine mesylate decreased expression levels of RAMP2 mRNA in both IMR-32 and NB69 cells. These findings indicate that adrenomedullin expression is induced during hypoxia in IMR-32 and NB69 neuroblastoma cells, but RAMP2 expression is rather suppressed under the same conditions. The decreased expression of RAMP2 and the ADM expression induction under hypoxia may constitute one mechanism of cellular adaptation to hypoxic stress.
UI - 11831928
AU - Wilson DJ; Dailey RA; Wobig JL; Dimmig JW
TI - Neuroblastoma within a congenital orbital teratoma.
SO - Arch Ophthalmol 2002 Feb;120(2):213-5
AD - 3375 SW Terwilliger Blvd, Casey Eye Institute, Portland, OR 97201-4197, USA. email@example.com
UI - 11747263
AU - Rohrer T; Trachsel D; Engelcke G; Hammer J
TI - Congenital central hypoventilation syndrome associated with Hirschsprung's disease and neuroblastoma: case of multiple neurocristopathies.
SO - Pediatr Pulmonol 2002 Jan;33(1):71-6
AD - Division of Pediatric Intensive Care and Pulmonology, University Children's Hospital Basel, Postfach, 4005 Basel, Switzerland.
We report on a male infant with the rare combined occurrence of congenital central hypoventilation syndrome (CCHS or Ondine's curse), Hirschsprung's disease (HD), and neuroblastoma. Current therapeutical options leave no doubt that children with isolated forms of CCHS, HD, or neuroblastoma must be treated, but management decisions and the ethical dilemma become more difficult with the presence of multiple neurocristopathies. Our patient was dependent on mechanical ventilation and total parenteral nutrition, when a neuroblastoma was diagnosed at age 5 months. We initiated an attempt at curative chemotherapy. The tumor failed to respond to recommended chemotherapeutic regimens, and the patient died at 11 months of age. We emphasize the importance of screening CCHS patients for associated illnesses such as neuroblastoma and ganglioneuroblastoma at time of diagnosis. Copyright 2002 Wiley-Liss, Inc.
UI - 11803583
AU - Maeda K; Matsuhashi S; Tabuchi K; Watanabe T; Katagiri T; Oyasu M; Saito
TI - N; Kuroda S Brain specific human genes, NELL1 and NELL2, are predominantly expressed in neuroblastoma and other embryonal neuroepithelial tumors.
SO - Neurol Med Chir (Tokyo) 2001 Dec;41(12):582-8; discussion 589
AD - Departments of Biochemistry and Neurosurgery, Saga Medical School, Saga, Japan.
NELL1 and NELL2 encode cysteine-rich amino acid sequences including six epidermal growth factor-like motifs, which contain signal peptides at the N-terminals. The deduced amino acid sequences of both genes are 55% identical and their cysteine stretch structures are conserved. NELL1 is expressed in the brain and kidney, whereas NELL2 is expressed specifically in the brain. The cell lineage expressing NELLs in the nervous system was investigated in established cell lines and central nervous system tumor tissues obtained from patients by Northern blot and reverse transcriptase-polymerase chain reaction analyses. NELL1 and NELL2 were predominantly expressed in neuroblastoma cell lines and little expressed in glioblastoma cell lines. NELL1 and NELL2 were also expressed in central neurocytoma, medulloblastoma, and some astrocytic tumors. Immunohistochemical analysis revealed that NELL2 protein was localized in the cytoplasm of neurons. These results suggest that NELL2 is predominantly expressed in the neuronal cell lineage in the human nervous system. NELL1 is expressed mainly in tumors in the neuronal cell lineage.
UI - 11801306
AU - Toraman AD; Keser I; Luleci G; Tunali N; Gelen T
TI - Comparative genomic hybridization in ganglioneuroblastomas.
SO - Cancer Genet Cytogenet 2002 Jan 1;132(1):36-40
AD - Department of Medical Genetics, School of Medicine, Akdeniz University, TR-07070, Antalya, Turkey.
The ganglioneuroblastoma are rare lesions with widespread neuronal differentiation that have been classified as intermediate stages between neuroblastoma and ganglioneuroma. To identify overall chromosome aberrations in ganglioneuroblastoma, we performed comparative genomic hybridization. All of the five tumor samples were found to exhibit multiple gains involving different chromosomal regions. Chromosomal gains displayed by chromosomes and chromosome loci were 2p25 approximately pter (60%), 5p15.1 approximately p15.3 (60%), 7 (60%), 13q22 approximately q31 (60%), and 22 (60%), which were detected as minimal common regions in all five tumor samples. Chromosome 22 gain, which had not been reported in neuronal tumors before, and novel site 13q22 approximately q31 may be considered to play an important role in progression and differentiation of ganglioneuroblastoma.
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