• Advances in pediatric surgery
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• v.6 no.2
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• pp.156-159
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• 2000

Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.11
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• pp.4671-4676
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• 2014

Background: MicroRNA-200a (miR-200a) has been reported to regulate tumour progression in several tumours but little is known about its role in neuroblastoma. Our aim was to investigate the potential role and mechanism of miR-200a in neuroblastomas. Materials and Methods: Expression levels of miR-200a in tissues were determined using RT-PCR. The effect of miR-200a and shAP-$2{\gamma}$ on cell viability was evaluated using MTS assays, and target protein expression was determined using Western blotting and RT-PCR. Luciferase reporter plasmids were constructed to confirm direct targeting. Results were reported as mean${\pm}$S.E.M and differences were tested for significance using the 2-tailed Students t-test. Results: We determined that miR-200a expression was significantly lower in neuroblastoma tumors than the adjacent non-cancer tissue. Over-expression of miR-200 are reduced cell viability in neuroblastoma cells and inhibited tumor growth in mouse xenografts. We identified AP-$2{\gamma}$ as a novel target for miR-200a in neuroblastoma cells. Thus miR-200a targets the 3'UTR of AP-$2{\gamma}$ and inhibits its mRNA and protein expression. Furthermore, our result showed that shRNA knockdown of AP-$2{\gamma}$ in neuroblastoma cells results in significant inhibit of cell proliferation and tumor growth in vitro, supporting an oncogenic role of AP-$2{\gamma}$ in neuroblastoma. Conclusions: Our study revealed that miR-200a is a candidate tumor suppressor in neuroblastoma, through direct targeting of AP-$2{\gamma}$. These findings re-enforce the proposal of AP-$2{\gamma}$ as a therapeutic target in neuroblastoma.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.259-264
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• 1985

Neuroblastoma is the most common extracranial solid tumor of childhood which presents various clinical symptoms depending on the primary and metastatic sites. However, it has been rarely reported that sudden onset of blindness was the chief complaint of neuroblastoma. A four years old boy was admitted to the Yeungnam University Hospital with the chief complaint of a sudden onset of blindness due to a distant metastasis of abdominal neuroblastoma to the sphenoid sinus. On admission, both side pupils were dilated without light reflex, fundoscopy showed pale optic disk, electroretinogram was subnormal and visual evoked potential showed no response. The liver was palpable in $3{\frac{1}{2}}$ finger breadth from the right costal margin and adult fist sized mass was palpable in the right flank. Skull X-ray showed destructed sphenoid bone and clinoid process and brain CT scan showed tumor mass in the sphenoid sinus and left orbit. Ultrasonogram and CT scan of the abdomen showed large tumor masses around the right kidney and para-aortic and retropancreatic lymph node. IVP showed displaced right calyceal system with preserved contour. Left supraclavicular lymph node which appeared after admission was biopsied and it showed poorly differentiated neuroblasts. He was treated according to the multiagent chemotherapy schedule for stage IV neuroblastoma patient of children's cancer study group. Abdominal tumor masses and sphenoid sinus mass were markedly reduced after 2 courses of the combination chemotherapy of cyclophosphamide, vincristine, DTIC, adriamycin and VM-26. Eventhough the blindness was not improved, the patient has been in good clinical condition.

• Journal of Korean Neurosurgical Society
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• v.49 no.1
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• pp.68-70
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• 2011

Neuroblastoma is a common tumor of children. We report a patient with extensive calvarial metastases of a neuroblastoma as an initial presentation. A 2-year-old girl presented with a history of gradually increasing head size and fever. A brain CT showed a multilobulated, large, extra-axial tumor involving both frontotemporoparietal areas with a sunray-spiculated hyperostosis of the skull and marked contrast enhancement. A brain MRI demonstrated extensive calvarial lesions with simultaneous involvement of the orbits. A biopsy was performed and a ganglioneuroblastoma was diagnosed. On systemic evaluation, an enlarged abdominal mass was detected. After neo-adjuvant chemotherapy, most of the tumors disappeared except for a tumor in the left parietal area; there was a corresponding decrease in the circumference of the head. We performed surgery for the remnant mass. Intensive chemotherapy was administered and a bone marrow transplantation was performed. Adequate neo-adjuvant chemotherapy followed by surgery to the neuroblatoma with extensive metastases to the skull and orbit may be helpful.

• The Korean Journal of Physiology and Pharmacology
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• v.2 no.4
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• pp.411-417
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• 1998

The role of phospholipase ($A_2\;PLA_2$) in tumor cell growth was investigated using SK-N-MC human neuroblastoma cells. 4-Bromophenacyl bromide (BPB) and mepacrine (Mep), known $PLA_2$ inhibitors, suppressed growth of the tumor cells in a dose-dependent manner without a significant cytotoxicity. Melittin (Mel), a $PLA_2$ activator, enhanced the cell growth in a concentration-dependent fashion. The growth-enhancing effects of Mel were significantly reversed by the co-treatment with $PLA_2$ inhibitors. In addition, Mel induced intracellular $Ca^{2+}$ release from internal stores like as did serum, a known intracellular $Ca^{2+}$ agonist in the tumor cells. Intracellular $Ca^{2+}$ release induced by these agonists was significantly blocked by $PLA_2$ inhibitors at growth-inhibitory concentrations. Arachidonic acid (AA), a product of the $PLA_2-catalyzed$ reaction, induced cell growth enhancement and intracellular $Ca^{2+}$ release. These effects of AA were significantly blocked by BAPTA/AM, an intracellular $Ca^{2+}$ chelator. Taken together, these results suggest that the modulation of $PLA_2$ activity may be one of the regulatory mechanisms of cell growth in human neuroblastoma cells. Intracellular $Ca^{2+}$ may act as a key mediator in the $PLA_2-induced$ growth regulation.

• Radiation Oncology Journal
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• v.37 no.3
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• pp.224-231
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• 2019

Purpose: To investigate the patterns of recurrence in patients with neuroblastoma treated with radiation therapy to the primary tumor site. Materials and Methods: We retrospectively analyzed patients with high-risk neuroblastoma managed with definitive treatment with radiation therapy to the primary tumor site between January 2003 and June 2017. These patients underwent three-dimensional conformal radiation therapy or intensity-modulated radiation therapy. A total of 14-36 Gy was delivered to the planning target volume, which included the primary tumor bed and the selected metastatic site. The disease stage was determined according to the International Neuroblastoma Staging System (INSS). We evaluated the recurrence pattern (i.e., local or systemic), progression-free survival, and overall survival. Results: A total of 40 patients with high-risk neuroblastoma were included in this study. The median patient age was 4 years (range, 1 to 11 years). Thirty patients (75%) had INSS stage 4 neuroblastoma. At the median follow-up of 58 months, there were 6 cases of local recurrence and 10 cases of systemic recurrence. Among the 6 local failure cases, 4 relapsed adjacent to the radiation field. The other 2 relapsed in the radiation field (i.e., para-aortic and retroperitoneal areas). The main sites of distant metastasis were the bone, lymph nodes, and bone marrow. The 5-year progression-free survival was 70.9% and the 5-year overall survival was 74.3%. Conclusion: Radiation therapy directed at the primary tumor site provides good local control. It seems to be adequate for disease control in patients with high-risk neuroblastoma after chemotherapy and surgical resection.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1279-1282
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• 2003

Neuroblastoma is the most common intraabdominal malignant tumor of childhood, with 40% arising from the adrenal gland. Bilateral adrenal involvement from synchronous development or metastatic spread of tumor is rarely seen in children with neuroblastoma. The patient was born with a spontaneous vaginal delivery. Birth weight was 3,200 g. Fetal ultrasonography showed a left adrenal cystic mass. At two weeks of age, she was admitted due to a massive abdominal distension and tachypnea. Percutaneous ultrasonography guided biopsy of the left adrenal mass was performed. The result of the biopsy was neuroblastoma. Vincristine and cyclophosphamide were administerd intravenously and 450 cGy of irradiation was added. Left adrenalectomy was accomplished and postoperative course was uneventful. The patient received cancer chemotherapy with a combination of carboplatin, ifosfamide and VP-16 and is now being followed up for three months. We have experienced a case of congenital bilateral neuroblastoma and report the case with brief review of related literatures.

• The Korean Journal of Cytopathology
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• v.6 no.2
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• pp.183-186
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• 1995

Fine needle aspiration has been effectively being applided to pediatric tumors since it renders a rapid diagnosis with minimal intervention. This measure is especially required for the large pediatric mass, which needs preoperative chemotherapy or radiotherapy to shrink the tumor to an operable size. A case of neuroblastoma of mediastinum, stage IV diagnosed by CT-guided FNA is described.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.sup1
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• pp.134-136
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• 2008

Neuroblastoma is the most common extracranial solid tumor in children. In diagnostic assessment of neuroblastoma, $^{18}F-FDG$ PET has been reported to have high diagnostic performance, especially, very high sensitivity in staging, restaging, and assessment of therapeutic efficacy. In comparison with conventional diagnostic imaging modalities including a, bone scan, and MIBG scan, $^{18}F-FDG$ PET showed better diagnostic performance. According to clinical research data hitherto, $^{18}F-FDG$ PET is expected to be an effective diagnostic tool in the management of neuroblastoma.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.1
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• pp.239-244
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• 2014

Several recent studies have showed that the n-myc downstream regulated gene 2 (NDRG2) is a new tumor suppressor gene, and that it plays an important role in tumor suppression in several cancers or cancer cell lines. However, few studies focused on its function in neuroblastoma cells. In the present investigation, we demonstrated that NDRG2 overexpression inhibited their proliferation. Using a cDNA microarray, we found that overexpression of NDRG2 inhibited the expression of cysteine-rich protein 61 (CYR61), a proliferation related gene. From our research, CYR61 may partially hinder NDRG2-mediated inhibition of cell proliferation. Overexpression of NDRG2 resulted in accumulation of cells in the G1 phase, which was accompanied by upregulation of p21 and p27 and downregulation of CDK4 and cyclin D1. Taken together, these data indicate that NDRG2 inhibits the proliferation of neuroblastoma cells partially through suppression of CYR61. Our findings offer novel insights into the physiological roles of NDRG2 in neuroblastoma cell proliferation, and NDRG2 may prove to be effective candidate for the treatment of children with neuroblastoma.