Question: This study aimed to elucidate the outcomes of high-risk neuroblastoma (HR-NB) patients treated with high-dose chemotherapy and stem cell rescue without immunotherapy. Finding: The 5-year overall survival and event-free survival rates were 45.1% and 40.4%, respectively. Meaning: High-dose chemotherapy plus stem cell rescue followed by cis-retinoic acid for 12 months is well tolerated and could improve survival in patients with HR-NB in limited resource settings. |
Although the prognosis is generally good in patients with intermediate-risk neuroblastoma, no consensus has been reached on the ideal treatment regimen. This study analyzed treatment outcomes and toxicities in patients younger than 18 months with stage 4 We retrospectively analyzed 20 patients younger than 18 months newly diagnosed with stage 4 |
Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid... |
To evaluate the potential utility of 123I-metaiodobenzylguanine (123I-MIBG) scintigraphy and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) for the detection of primary and metastatic lesions in pediatric neuroblastoma (NBL) patients, and to determine whether 18F-FDG PET is as beneficial as 123I-MIBG imaging. We selected 8 NBL patients with significant residual mass after operation and who had paired 123I-MIBG and 18F-FDG PET images... |
Although high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT) have improved the prognosis for patients with high-risk neuroblastoma (NB), event-free survival rates remain in the range of 30 to 40%, which is unsatisfactory. To further improve outcomes, several clinical trials, including tandem HDCT/autoSCT, high-dose 131I-metaiodobenzylguanidine treatment, and immunotherapy with NB specific antibody, have been undertaken and pilot studies have reported... |
Purpose : The purpose of this study was to evaluate the clinical characteristics and outcomes of patients with neuroblastoma aged less than 1 year. Methods : From January 1997 to December 2007, 41 patients aged less than 1 year were diagnosed with neuroblastoma. Patients were divided into 3 risk groups according to the stage of the disease and N-myc amplification. Low-risk... |
Neuroblastoma is one of the most common extracranial solid tumor of childhood, and treatment of refractory neuroblastoma remains a significant clinical problem. Iodine-131-metaiodobenzylguanidine (131I-MIBG) therapy is an alternative approach to treat stage Ⅳ neuroblastoma. We report the palliative effect of 131I-MIBG in three cases of relapsed neuroblastoma after autologous peripheral blood stem cell transplantation. 131I-MIBG is an effective and relatively... |
Purpose : α-Galactosylceramide (α-GalCer)-stimulated human Vα24 natural killer T (NKT) cells exert antitumor activity against some leukemia in a CD1d dependent and TCR-mediated manner, but could not kill CD1d - negative neuroblastoma (NB) cells. There are few reports about the direct antitumor effect of highly secreted cytokines by these cells on activation. In this study, using a cell-free supernatant (SPN)... |
Purpose : In order to assess the usefulness of serum ferritin as a marker of disease activity and prognostic factor in pediatric malignancy, serum ferritin levels were measured. Methods : Peripheral blood samplings for ferritin level were made at presentation, in remission following therapy, and in relapse in 95 children with malignancy admitted to the Department of Pediatrics, Kosin University Gospel... |
Purpose : Recent clinical studies have shown that arsenic trioxide(As2O3) at low concentrations induces complete remission without significant toxicity in patients with refractory acute promyelocytic leukemia(APL). Like APL cells, neuroblastoma(NB) cells are thought to be arrested at an early stage of differentiation, and can respond with retinoic acid treatment. Sulindac, a nonsteroidal antiinflammatory drug, was reported to induce antitumor activity... |
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... |
Retinoic acid has been used successfully as a differentiating agent in acute promyelocytic leukemia and neuroblastoma. However, some adverse effects have been recognized, such as headaches, dry skin and retinoic acid syndrome, a life threatening acute cardiorespiratory disorder. Acute pancreatitis with hyperlipidemia has rarely been reported. We experienced a case of acute pancreatitis with hyperlipidemia in a neuroblastoma patient after... |
Purpose : Neuroblastoma is a malignant neoplasm which arises from primitive sympathetic neuroblasts, and occasionally can matured from a malignant neuroblastoma into a benign ganglioneuroma. It has the highest rate of spontaneous regression of any pediatric tumor. We performed a retrospective study of pathologic features after combination therapy in advanced neuroblastoma. Prognostic effects of the individual morphologic feature and prognostic... |
Purpose : IFNγ sentitizes many tumor cells to TNFα and FASL-mediated apoptosis by enhancing the expression of TNF or FAS/CD95 receptor and modulating the activation of caspase and Bcl-2 family. It has been reported that IFNγ and TNFα synergistically caused differentiation and growth inhibition of neuroblastoma cells. Even though some neuroblastoma cell express FASR/FASL on the cell surface, they could... |
Purpose : Selective introduction of genes conferring chemosensitivity into proliferating tumor cells may be used to treat cancer. We first investigated the bystander effect of retrovirus-mediated gene transfer of herpes simplex virus thymidine kinase(HSV-TK) gene to murine neuroblstoma cell line(neuro-2a) in vitro and in vivo. Second, we examined the mechanism and its enhancement of the bystander effect in murine neuroblastoma. Methods... |
Purpose : To demonstrate the bystander effect in murine neuroblastoma model which transduced with HSV-TK gene in vitro and in vivo. Methods : The LNC/TK vector was transfered in vitro into the neuro-2a cells, murine neuroblastoma cell line. Variable mixed populations of neuro-2a cells consisting of HSV-TK+ or HSV-TK- were plated into culture plates and treated with GCV for... |
Purpose : Interleukin(IL)-16 is a potent chemoattractant factor for CD4+ T cells, monocytes, and eosinophils. It up-regulates IL-2R on CD4+ T lymphocytes and regulates the function of antigen presenting cells. We used retrovirus-mediated gene transfer of the human IL-16 gene into the neuro-2a cells, which is the murine neuroblastoma cell lines, to investigate whether locally secreted IL-16 might generate anti-tumor... |
The authors report a 7-year-old boy with neuroblastoma complicated by severe hypertension and pulmonary edema. Abdominal computed tomographic scan revealed a huge mass surrounding the aorta. After administration of cancer treatment, there was a marked increase in serum catecholamines level and hypertension, which resulted in pulmonary edema and heart failure. Alpha adrenergic blocking agents(prazocin, terazocin) were administrated, successfully controlling the... |
Purpose : Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage Ⅲ or Ⅳ of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma. Methods : Twenty-nine children diagnosed with stage Ⅲ or Ⅳ neuroblastoma after 1 year of age, between May... |
Background : Gene transfer with vectors derived from murine retroviruses is restricted to cells which are proliferating and synthesizing DNA at the time of infection. Accordingly, selective introduction of genes encoding for susceptibility to otherwise nontoxic drugs(suicide genes) into proliferating tumor may be used to treat cancer. We investigated the efficacy of in vitro transduction of neuroblastoma cell with the herpes simplex-thymidine kinase(HSV-tk) gene followed... |
Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have been associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result... |
Neuroblastoma is one of the most common tumors of childhood. It accounts for about 8% of all pediatric cancers. Because most children who present with this tumor are older than 1 year of age and have advanced disease, they have a poor prognosis in spite of intensive treatment. The patients with less than 1 year of age at the time of... |
As childhood cancer survivors are now increasing in number owing to the improvements in diagnosis, staging, and treatment, concerns have been raised regarding the quality of survival of these patients. One of the limitation to quality and length of survival has been the development of second malignant neoplasm (SMN). In Korea, only one case of SMN has been reported. However, one can expect that... |
Metastatic neuroblastoma to whole bones and brain generally has been considered incurable, but some reports have been published with good and partial response after initial chemotherapy followed by second-look operation. But the long term survival is dismal. Recently we experienced a case of metastatic neuroblastoma to brain without neurologic symptoms confirmed by computed tomograpy, who had multiple bony metastases including skull. The whole metastatic... |
Ganglioneuroblastoma is a malignant tumor which contains characteristics of both the neuroblastoma and the ganglioneuroma. It may be invasive and metastasized to the distant organs. We experienced a case of ganglioneuroblastoma which was arised from the left adrenal gland and metastasized to the left femur and the bone marrow. A 3 year old male patient was diagnosed as ganglioneuroblastoma and confirmed histologically A review of literatures... |
Neuroblastoma is one of the most common malignancy in infancy & childhood, but congenital neuroblastoma is rare. We experienced a case of congenital neuroblastoma of right posterior mediastinum without metastasis in 10-day-old female neonate. The diagnosis was established by autopsy including gross and microscopic finding. A brief review of literatures was made. |
17 Cases of neuroblastoma diagnosed and treated at the Kosin Medical College from Mar. 1978 toFeb. 1985 were analyzed and the results are as follows: 1) Ages at the time of diagnosis were from 7 months to 9 years. The mean age was 3 10/12 years. 29.4% of the patients were below the age of 2 and 82.3% of the... |
Neuroblastoma is one of the most common malignant tumors in infants and children, which arises from sympathetic nervous system. We had experienced a case of neuroblastoma originated from mediastinum and metastized to bone & lymph node. |
Neuroblastoma is the commonest malignant tumor identified during the neonatal period and the majority of neuroblastomas are felt to be congenital in origin. Neuroblastoma is a tumor of particular interest because it is known to arise from cells of the neural crest which possess the potential for maturation of spotaneous regression. This report present the clinical, radiographic and autopsy findings of... |
Congenital unilateral renal agenesis is a rare congenital anomaly. Furthermore, renal agenesis associated with neuroblastoma and cryptorchism is more rare in incidence. Recently we experieced a case of Rt. renal agenesis combined with cryptorchism and neuroblastoma. We diagnosed it with IVP and selective renal angiography and confirmed it with explolaparotomy and biopsy. We reviewed the literatures on renal agenesis, cryptorchism... |