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)

The selective uptake and accumulation of 131J-Metaiodobenzylguanidine in neuroblastoma cells in vivo may be utilized for targeted irradiation. The experience with 32 neuroblastoma patients refractory to conventional high dose chemotherapy is reported. At diagnosis 8 patients had Evans stage III and 22 stage IV. 11/32 experienced recurrences after complete tumor disappearance and before mIBG treatment, 16/32 progressed from residual or nonresponding tumor and in 3/32 insufficient tumor regression by chemotherapy was observed. 2 children received one mIBG course each with no evidence of disease. Mean applied activity was 128 mCi per course (35-300 mCi), 360 mCi per patient (80-1033 mCi) and 19.2 mCi/kg per patient (3.2-37.9 mCi/kg), respectively. A total of 84 courses was given (mean 2.6 per patient). Pain relief was noticed in 14/14 patients with bone pain. Complete or very good partial remission was achieved in 5/32, partial remission in 11/32 and stable disease in 6/32 patients. In 8 children progression occurred and 2 patients were not evoluable. 20 children died, 12 are still alive (6 patients with initial stage IV, 6 with stage III disease). Main side effect was transient thrombocytopenia, which became more severe with increasing number of courses. We conclude that mIBG treatment is effective in some patients with refractory neuroblastoma and may be utilized in the future as front line therapy for patients achieving only incomplete regressions after high dose chemotherapy.
 ...
PMID:[131(I)-meta-iodobenzylguanidine treatment of 32 children with therapy-refractory neuroblastoma]. 306 60

A prospective study of the effectiveness of ifosfamide as a single agent in the management of previously untreated patients with Evans stage IV neuroblastoma was undertaken. Eighteen children aged more than 1 year were treated with ifosfamide (IFX) 3 g/m2 daily for 2 days immediately after diagnosis and 3 weeks later. Treatment was continued with combination chemotherapy using vincristine, cyclophosphamide, cisplatinum and etoposide (OPEC) or a variant. Mesna (2-mercaptoethane sulphonate) was given to all patients during IFX treatment to prevent urotoxicity. Eight of the 18 patients (44%) responded to IFX. Nine had greater than 66% reduction in baseline tumor volume. Of 15 evaluable patients with raised pre-treatment urinary catecholamine excretion, six (40%) achieved greater than 50% reduction in pretreatment levels. Two of 10 patients evaluable for bone marrow response had complete clearance. Toxicity was mild in all patients. Upon completing 'first line' therapy, only four patients (22%) achieved a good partial remission (GPR) or complete response (CR). Median survival was 11 months. There was a lower rate of attaining GPR and shortened median survival in patients receiving phase II IFX before OPEC or variant, compared to patients with similar pre-treatment characteristics treated with OPEC from diagnosis in an earlier study.
 ...
PMID:Ifosfamide in previously untreated disseminated neuroblastoma. Results of Study 3A of the European Neuroblastoma Study Group. 316 95

Patients with widespread neuroblastoma (NB) frequently have elevated serum ferritin levels, and recently anti-NB effects of the iron chelator deferoxamine (DFO) have been reported. We have investigated the effect of DFO on human bone marrow NB cells from two untreated children with Evans Stage IV disease. DFO treatment caused dose- and time-dependent cytotoxicity of NB cells, with maximal killing at exposure to 50 micron DFO for 72 h. Cytotoxicity was prevented by cotreatment with stoichiometric amounts of iron salts and reversible by removal of DFO or addition of iron salts within 48 h of treatment. Additionally, DFO inhibited clonal growth of human bone marrow NB cells in methylcellulose in a time- and dose-dependent manner. These effects were also prevented by cotreatment with iron salts. Thus, DFO has potent antitumor effects on human NB cells which appear to be related to iron deprivation. DFO should be considered for further preclinical evaluation as an anti-NB agent.
 ...
PMID:Deferoxamine inhibition of human neuroblastoma viability and proliferation. 319 93

Fifteen children with advanced neuroblastoma according to Evans' classification (1 with stage III and 14 with stage IV) were treated with high-dose melphalan (HDM) followed by autologous bone marrow transplantation. Before HDM, all patients had been extensively treated with multimodality therapy for a median duration of 9 months. At the time of HDM, seven children were in partial remission (PR) with measurable residual tumor and 8 were in complete remission (CR) or good partial remission (GPR). No reduction in measurable tumor size was observed in any of the PR patients. However, when HDM was used as consolidation therapy (CR and GPR patients) survival appeared encouraging, since five of eight patients are alive with no evidence of disease at (NED) 29+ to 54+ months after HDM. Tolerance of this high-dose chemotherapy was satisfactory; gastrointestinal toxicity appeared to be the most important limiting factor. These results suggest that chemotherapy including high-dose melphalan is promising when used as consolidation therapy in patients who have already attained CR with conventional therapies.
 ...
PMID:Treatment of advanced neuroblastoma with high-dose melphalan and autologous bone marrow transplantation. 351 13

A newborn with symptoms of massive adrenal hemorrhage is presented. The cause was a unilateral neuroblastoma of the adrenal gland, bringing about severe anemia and hemorrhagic shock. The suspicion of a tumor of the adrenal gland was confirmed by infusion urography, ultrasound examination of the kidney, and the increased amount of vanillymandelic acid in the urine. Nephrectomy was performed on the fourth day of the newborn's life. The tumour was classified in the IV-S stage after Evans et al. Following the operation, neither cytostatics nor radiation was applied. The child is developing normally and the amount of vanillymandelic acid in the urine is within normal limits. The child is now in the 16th month of life.
 ...
PMID:[Massive adrenal hemorrhage in a neonate due to neuroblastoma]. 356 Oct 28

Neuroblastoma (NB) is a tumor usually arising in children and young adults showing different degrees of malignancy. Recently, the presence of N-myc amplification in neuroblasts has been associated with a poor outcome in the late stages of disease (Evans's Stage IV). Until now no amplification of N-myc gene has been observed in Stage IV-S, usually considered to have a favorable prognosis. In this paper we report a case of a child affected by NB Stage IV-S showing mild N-myc gene amplification. The finding of N-myc amplification in our patient shows that such alteration of the N-myc oncogene is not necessarily correlated with a poor prognosis; in this light the role of N-myc amplification in the neoplastic process should be reconsidered.
 ...
PMID:N-myc oncogene amplification in a patient with IV-S neuroblastoma. 359 19

The treatment of neuroblastoma must take into account the prognosis as determined at the time of diagnosis. The stage is an important prognostic factor. In Evans's Stage I neuroblastoma, surgery is curative in itself and adjuvant treatment is not needed. In Evans' Stage II, it is our opinion that the presence of involved lymph nodes worsens the prognosis. Therefore, we suggest that patients with Stage II should be divided into Stage IIA (without lymph node involvement) and Stage IIB (with lymph node involvement). The treatment of Stage IIA patients may be the same as for Stage I patients, i.e., surgery alone. However, Stage IIB patients should be treated with intensive combined chemotherapy of short duration following surgery.
 ...
PMID:Treatment of localized neuroblastoma. 376 13

Neuroblastoma, a malignant tumor of neural crest origin, is the most common extracranial solid tumor in children. In 1971 Evans et al. introduced a clinical staging for neuroblastoma. Over sixty percent of patients present with neuroblastoma beyond stage I. Despite more aggressive therapy there has been only minimal improvement in survival. Since 1978, all patients with neuroblastoma have had CT scanning as part of their initial evaluation at our institution. Children with abdominal neuroblastoma beyond stage I form the basis of this report. Selected cases illustrating the permeative nature of neuroblastoma and the mechanism of direct abdominal spread by CT scanning are presented. The tumor originates in the retroperitoneum and spreads to the abdominal aorta where it gains access to the subperitoneal space via the celiac axis and superior mesenteric artery. These vessels course from the aorta to their ultimate destination within their peritoneal folds. These folds form the interconnecting space (subperitoneal space) between the retroperitoneum and the peritoneal organs. Such scanning is extremely sensitive in detecting neuroblastoma with early infiltration into adjacent tissues and contiguous spread through abdominal spaces. The clinical implications of the permeative nature of neuroblastoma and the mechanism of contiguous abdominal spread are discussed.
 ...
PMID:Mechanism of direct spread of abdominal neuroblastoma: CT demonstration and clinical implications. 379 60

Among children over 1 year of age with Evans Stage IV neuroblastoma, there appears to be a small group with a relatively favorable prognosis. These patients have extensive lymph node metastases (cervical/axillary/thoracic/abdominal/pelvic), but no extranodal metastases. Three of six such patients (50%) are long-term disease-free survivors, compared with none of 40 patients with extranodal metastatic disease (p less than 0.0002). Patients with only lymph node metastases (Stage "IV-N") may have a biologically more favorable tumor that is curable with conventional, intensive multimodality therapy.
 ...
PMID:Stage IV-N: a favorable subset of children with metastatic neuroblastoma. 401 Jun 21

There is considerable controversy regarding the role of radiation therapy in the treatment of neuroblastoma. Postoperative irradiation in the range of 2500-4000 rad is commonly used in the treatment of Evans Stage II or III disease, but there are no data in the literature to suggest the optimum dose of radiation that is necessary. Because much lower doses have been used at the University of Florida, a retrospective study was undertaken in an attempt to determine the optimum dose necessary in conjunction with surgery. From March 1964 through July 1979, 21 children with Stage II or III neuroblastoma were seen at the University of Florida. One patient died postoperatively. The remainder received postoperative irradiation with doses ranging from 900 to 4500 rad. The lower dose of radiation used did not adversely influence survival, particularly for patients less than two years of age at diagnosis. In this group, no patient had a local recurrence or died of disease, even though nine of 15 available patients received doses of 900-1500 rad.
 ...
PMID:Pediatric neuroblastoma: postoperative radiation therapy using less than 2000 rad. 640


<< Previous 1 2 3 4 5 Next >>