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.
Biopsy, Fine-Needle* ; Diagnosis ; Drug Therapy ; Mediastinum* ; Neuroblastoma* ; Radiotherapy

So far treatment of advanced neuroblastoma is still difficult, due to its high malignancy. Currently comprehensive therapies, including high-dose multi-drug chemotherapy, surgery, stem cell transplantation, radiation, biological therapy and immune therapy as well as target therapy dominant the treatment of this disease, and we hereby introduce the latest development of treatment protocols for this disease.
Combined Modality Therapy ; Female ; Humans ; Male ; Neoplasm Recurrence, Local ; therapy ; Neuroblastoma ; therapy

We used retroviral-mediated gene transfer of the human interleukin (IL)-2 gene into murine neuroblastoma cells to investigate whether locally-secreted IL-2 is able to influence the generation of anti-tumor immune responses. Supernatant obtained from cultures of approximately 1 x 10(6) IL-2 gene-transduced, G-418 selected neuro-2a cells was assayed for human IL-2 production by ELISA kit. First, to estimate whether the local secretion of IL-2 from the genetically-modified tumor cells would affect their tumorigenicity in vivo, IL-2-secreting neuro-2a cells were s.c. injected into A/J mice and tumor growth was measured weekly. And to estimate whether IL-2 transfected neuroblastoma cells protect mice from tumor development after wild-type tumor cell challenge, IL-2-secreting neuro-2a cells were s.c. injected into A/J mice. Seven days after IL-2 gene-transfected neuroblastoma cell injection, unmodified neuro-2a cells were s.c. injected into the contralateral site of A/J mice and tumor growth was measured weekly. Finally, to estimate IL-2 effect on pre-established large tumor burdens, IL-2-secreting neuro-2a cells were s.c. injected into A/J mice with established tumor and its growth was measured weekly. The IL-2 gene-transduced neuro-2a clones secreted 120.25-177.3 IU of IL-2 per ml per 10(6) cells during 24 hr. None of the mice injected with IL-2-secreting neuro-2a cells developed tumors within 6 weeks, while all of the mice injected with wild-type neuro-2a cells developed tumors. Immunization of mice with IL-2 gene-transfected, irradiated neuro-2a cells protected these animals against a subsequent challenge with wild-type tumor cells. Finally, the size of large neuroblastomas decreased after IL-2-secreting neuro-2a cell injection into mice. Local secretion of IL-2 gene-transduced tumor cells abrogates their tumorigenicity and induces protective immunity and may inhibit the growth of neuroblastoma.
Animal ; Antibody Formation ; Gene Transfer Techniques* ; Human ; Immunization/methods ; Interleukin-2/therapeutic use ; Interleukin-2/genetics* ; Mice ; Neoplasm Transplantation ; Neuroblastoma/therapy ; Neuroblastoma/prevention & control ; Neuroblastoma/pathology ; Neuroblastoma/genetics* ; Retroviridae/genetics* ; Tumor Cells, Cultured

Intensive chemotherapy with or without stem cell rescue has become widely accepted for treatment of neuroblastoma because increased dose-intensity correlates with improved response rates. For neuroblastoma that is resistant to intensive chemotherapy, further use of high-dose therapy is unlikely to be beneficial. For disease that recurs after myeloablative consolidation, high-dose salvage therapy may not be feasible because of poor bone marrow reserve and may not be justified in view of its morbidity in the absence of a realistic chance for cure. One treatment option in these difficult clinical settings is chronic oral administration of low-dose etoposide. However, there has been a few clinical reports for the experience of oral etoposide for refractory neuroblastoma. We now present 2 cases of successful response of oral etoposide for refractory neuroblastoma.
Administration, Oral ; Bone Marrow ; Drug Therapy ; Etoposide* ; Neuroblastoma* ; Salvage Therapy ; Stem Cells

OBJECTIVETo investigate the clinical features and outcomes of neuroblastoma (NB) children aged above 5 years, and to provide a theoretical basis for improving prognosis.

METHODSA retrospective analysis was performed for the clinical data of 54 previously untreated NB children, and their clinical features and outcome were analyzed. The Kaplan-Meier method was used for survival analysis.

RESULTSAmong the 54 children, there were 36 boys and 18 girls, and all of them had stage 3 or 4 NB. Of all the children, 41 (41/54, 76%) had retroperitoneal space-occupying lesions, 10 (10/54, 18%) had mediastinal space-occupying lesions, 2 (2/54, 4%) had intraspinal space-occupying lesions, and 1 (1/54, 2%) had pelvic space-occupying lesions. At the end of the follow-up, 30 children (30/54, 56%) survived, among whom 23 (77%) achieved disease-free survival (9 achieved complete remission after chemotherapy for recurrence), 6 (20%) achieved partial remission of tumor (all of them received chemotherapy again due to recurrence), and 1 (3%) experienced progression (with progression after chemotherapy again due to recurrence); 24 children (44%) died, among whom 22 died after chemotherapy again due to recurrence and 2 died of multiple organ failure during the first treatment. According to the Kaplan-Meier survival analysis, the mean survival time was 53.8 months, and the children with stage 3 NB had a significantly higher overall survival rate than those with stage 4 NB (80% vs 53%; p<0.01). The children with recurrence had a significantly lower mean survival time than those without recurrence (51.68 months vs 62.57 months; p<0.01).

CONCLUSIONSOlder children often have late-stage NB, but standard treatment can improve their outcomes.

Adolescent ; Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Humans ; Male ; Neuroblastoma ; mortality ; therapy ; Retrospective Studies

OBJECTIVETo investigate the clinical features, treatment modalities and the prognosis of advanced neuroblastoma in children.

METHODSThe medical records of 63 children with stage III or IV neuroblastoma from January 1996 to December 2005 were retrospectively reviewed. Sixty patients were treated by tumor resection and (or) chemotherapy and (or) radiation. Fourteen out of the 60 patients received another autologous peripheral blood stem cell transplantation.

RESULTSOf the 63 patients with advanced neuroblastoma, the male/female ratio was 2.7:1 and the median age at diagnosis was 4 years old. Most of the initial symptoms included pyrexia, abdominal pain, abdominal mass, and leg or articular pain. Primary tumor sites were adrenal (38%), retroperitoneal (35%), mediastinal (17%), pelvic (6%) and cervical (2%). The sites of metastasis at diagnosis included local (41%) and (or) distant (37%) lymph nodes, bone marrow (60%), bone (46%) and liver (16%). The median survival time of the 63 patients was 32.7 months. The 2-year survival rate was 44.3%. Statistical analysis demonstrated that unfavorable survival prognostic factors were the following: age > 1 year at diagnosis (P < 0.05); serum neuro-specific enolase > 100 mg/L (P < 0.05); serum lactic dehydrogenase > 1500 U/L (P < 0.01); serum ferritin >150 mg/L (P < 0.05). The overall survival period of the patients was prolonged through total resection of the primary tumor (P < 0.05). Intensive chemotherapy in combination with autologous peripheral blood stem cell transplantation could also result in a prolonged overall survival period (P < 0.01).

CONCLUSIONSNeuroblastoma with advanced stages often presents with various clinical manifestations and has a poor prognosis. It is beneficial to improve the prognosis of neuroblastoma through an early diagnosis and a comprehensive therapy including total resection of the primary tumor, autologous peripheral blood stem cell transplantation and intensive chemotherapy.

Adolescent ; Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Humans ; Infant ; Male ; Neuroblastoma ; mortality ; therapy ; Prognosis

Autologous stem cell transplantation (ASCT) for the treatment of high-risk neuroblastoma (NBL) is an accepted method for restoring bone marrow depression after high dose chemotherapy. We retrospectively analyzed eighty eight cases of NBL that underwent ASCT following marrow ablative therapy at 12 transplant centers of the Korean Society of Pediatric Hematology-Oncology between January 1996 and September 2000. Seventy nine children were of stage IV NBL and 9 were of stage III with N-myc amplification. Various cytoreductive regimens were used. However, the main regimen was 'CEM' consisting of carboplatin, etoposide and melphalan, and this was used in 66 patients. Total body irradiation was also added in 36 patients for myeloablation. To reduce tumor cell contamination, stem cell infusions after CD34+ cell selection were performed in 16 patients. Post-transplantation therapies included the second transplantation in 18 patients, interleukin2 therapy in 45, 13-cis retinoic acid in 40, 131-meta-iodobenzylguanidine in 4, conventional chemotherapy in 11, and local radiotherapy in 8. Twenty two patients died, sixty six patients are surviving 1 to 46 months after ASCT (median followup duration, 14.5 months). Although the follow-up period was short and the number of patients small, we believe that ASCT might improve the survival rate in high-risk NBL.
Adolescent ; Child ; Child, Preschool ; Combined Modality Therapy ; Female ; Human ; Korea ; Male ; Myeloablative Agonists/therapeutic use ; Neuroblastoma/mortality ; Neuroblastoma/pathology ; Neuroblastoma/therapy* ; Retrospective Studies ; Stem Cell Transplantation* ; Survival Rate ; Transplantation Conditioning ; Transplantation, Autologous ; Treatment Outcome

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 groupings according to modified Shimada classification systems were analyzed. METHODS: The treatment results for six patients with neuroblastoma seen at Keimyung University from Jan. 1991 to June 2000 were analyzed. Patients were treated with a combination of chemotherapy, radiation therapy, and surgery, and classified by two major prognostic criteria based on morphological features of neuroblastoma, such as modified Shimada classification and histologic grading. RESULTS: Three cases were classified to a good histologic group; among them, two cases survived, but one case was lost in follow-up. There were three cases classified in a poor histologic group. All of these patients expired due to sepsis and hemorrhagic pancreatitis. CONCLUSION: Prognostic classification due to pathologic findings had significant value in evaluating the survival rate of neuroblastoma patients.
Classification ; Drug Therapy ; Follow-Up Studies ; Ganglioneuroma ; Humans ; Neuroblastoma* ; Pancreatitis ; Prognosis* ; Retrospective Studies ; Sepsis ; Survival Rate

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 another 4 days. Surviving cells were counted and cell viability was determinated. For investigating the in vivo bystander effect, variable mixed populations of neuro-2a cells consisting of HSV-TK+ and HSV-TK- were inoculated into A/J mice. The tumor size was measured following injection of GCV for 7 days. RESULTS: The survival rate of the 100% neuro-2a/TK group was 90%, 25%, 5% and 0%, of 50% neuro-2a/TK group was 92%, 30%, 10% and 0%, and of the 10% neuro-2a/TK group was 95%, 40%, 15% and 5%. But, the survival rate of 0% neuro-2a/TK group was 120%, 150%, 180% and 220% on days 1, 2, 3, and 4 respectively. In the 100% and 50% neuro-2a/TK groups, tumor had disappeared following administration of GCV and in 10% neuro-2a/TK group, tumor size was not increased during GCV treatment. In 0% neuro-2a/TK group, tumor size increased during administration of GCV and all mice died after 6 weeks. CONCLUSIONS: We demonstrated the bystander effect in a murine neuroblastoma model which transduced with HSV-TK gene in vitro and in vivo. These results suggest that HSV-TK/GCV gene therapy may be useful for treatment of neuroblastoma.
Animals ; Bystander Effect* ; Cell Line ; Cell Survival ; Genetic Therapy* ; Mice ; Neuroblastoma* ; Survival Rate

Neuroblastoma stage IV-S patients have frequent spontaneous remission and high survival rate. Many investigators have recommended minimal or no therapeutic intervention ; however, some patient do experience progressive disease and ultimately die of neuroblastoma. We experienced a case of stage IVS neuroblastoma with N-myc amplification and coagulopathy. This patient has treated with combination chemotherapy and radiation therapy, then remained disease free for 1 year on the follow up till March, 1997.
Drug Therapy, Combination ; Follow-Up Studies ; Humans ; Neuroblastoma* ; Remission, Spontaneous ; Research Personnel ; Survival Rate