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)

We have been among the first authors to point out that false negative cases could be observed with 131I-MIBG scintigraphy for neuroblastoma. We have observed until now ten of such false negative cases, 7 with primary tumor and 3 with bone metastases. Fifty 131I-MIBG scans were performed in 35 children with histologically proven neuroblastoma (24 grade IV) and compared to bone scans, CT and NMR images, ultrasound and clinical results. The visualization of the primary tumor shows a higher sensitivity with MIBG (79%) than with bone scans (47%) and a 100% specificity with each method. MIBG and bone scans, for bone metastases, are similar in the sensitivity (87.5%) but MIBG is much more specific (100%) than bone scan (81%). These results clearly confirm the superiority of MIBG scan for detection of primary tumor as well as bone metastases. However, MIBG is not always the most appropriate investigation, as shown by 11 observed pitfalls. Ten false negative cases have been observed and must be considered: in five out of 10 cases, bone scans performed with 99m Tc-HMDP made the diagnosis (3/7 cases of primary tumor and 2/3 cases of bone metastases). Moreover, one case was not usable due to a large digestive uptake. Our aim is to understand the reasons of the false negative by a meticulous analysis of every single case. The optimal procedure for neuroblastoma diagnosis, extent and follow up clearly seems to be the following strategy: MIBG scan must be firstly performed; in case of non-demonstrative scan the bone scan, which is complementary, will greatly contribute to the diagnosis.
 ...
PMID:Pitfalls and solutions in neuroblastoma diagnosis using radioiodine MIBG: our experience about 50 cases. 326 25

From 1983 to 1986, 21 patients with poor prognosis neuroblastoma were treated with bone marrow transplantation. This regimen included induction chemotherapy, delayed surgical resection, local irradiation, and intensive chemoradiotherapy followed by infusion of allogeneic or autologous marrow. This therapeutic approach resulted in a 57% long-term survival rate (follow-up: 14-48 months), which appears to be approximately three times superior to conventional chemotherapy in a comparable group of children. In addition, complete resection was possible in 11 of 17 patients operated on after induction therapy. Recurrence in the primary site after bone marrow transplantation occurred in only one of 18 evaluable patients. Thus, this approach almost always eradicates primary tumor in patients with neuroblastoma with advanced disease.
 ...
PMID:Delayed surgery and bone marrow transplantation for widespread neuroblastoma. 331 Sep 32

The clinical presentation of the disease and the results of treatment in 42 patients with malignant peripheral neuroectodermal tumors (MPNT) entered into the Cooperative Ewing's, soft tissue, and neuroblastoma trials of the German Society of Pediatric Oncology were retrospectively analyzed. Within the Ewing's sarcoma trial, patients with chest wall lesions were particularly analysed for MPNT features. The period of observation ranged from 15 to 86 months; the median relapse-free time was 24 months. There were 28 male and 14 female patients, the median age of patients was 15 years (range, 9 months-23 years). Thirty-two patients had localized disease (M0), and ten patients presented with primary metastases (M1). The predominant location of the tumors was the thoracopulmonary region, followed by the extremities, the abdominal/pelvic, and head and neck region. Thirty-one of 42 tumors involved the adjacent bone. The disease-free survival according to Kaplan-Meier life-table analysis was 56% +/- 11% for Stage M0 patients at 3 years. Nine of ten patients with M1 disease showed progression of their disease. Most patients had combined modality treatment with surgery, chemotherapy and radiation therapy. Best results were obtained with extensive surgery. Radiation doses ranged from 20 to 60 Gy and could not be correlated with the outcome of the disease. Most recurrences occurred at the site of the primary tumor. In patients with primary chemotherapy after biopsy-proven diagnosis, the responsiveness of this disease to chemotherapy could be demonstrated. Combination chemotherapy containing anthracyclines and high doses of alkylating agents appeared superior.
 ...
PMID:Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. 333 70

The following study was performed to investigate whether antitumor agents can induce maturation of advanced neuroblastoma (NB). Of 56 NB patients (1970-1987), 13 in stage III or IV underwent resection of the primary tumor (11 abdominal, 2 mediastinal) following complete or partial response to preoperative chemotherapy (11 conventional cytotoxic chemotherapy, 2 conventional cytotoxic chemotherapy combined with intraarterial infusion of prostaglandin E1 and oral papaverine). Histological examinations, performed on these 13 NB patients before and after chemotherapy, showed (1) differentiation of the NB to ganglioneuroblastoma (GNB) with predominance of mature atypical ganglion cells, development of stromal components and a few immature malignant cells in 10 of 13 and (2) differentiation of the NB to a more mature form than was seen histologically before treatment in the remaining 3. Our results indicate that the morphological differentiation of immature NB in advanced stage to more mature GNB can be induced by conventional cytotoxic chemotherapy with or without so-called 'maturation-inducing' agents. However, the chemotherapy-induced morphological differentiation did not correlate with the prognosis of the patients.
 ...
PMID:Neuroblastoma: incomplete differentiation on the way to maturation or morphological alteration resembling maturity? 336 91

Clinical and biological significance of increased urinary excretion of dopamine in Japanese children with neuroblastoma was investigated. There was an increase in dopamine excretion in 19 of 29 patients (66%) and 15 of 19 in stages III and IV (79%). When the ratio of noradrenaline and dopamine was divided into two at the value of 3.5 x 10(-2), the disease-free survival rate was four of 16 (25%) in the low ratio group and nine of 19 (69%) in the high ratio group. In five patients, the urinary analysis revealed that only the level of dopamine was elevated before initiation of the therapy. The common features of these patients were as follows: (1) the age at diagnosis was 1 to 4 years; (2) all originated from the suprarenal region; (3) stages were advanced III or IV; and (4) the prognosis was poor. N-myc oncogene of the primary tumor was evident in three, and all were amplified to 32, 37, and 112 copies. These observations suggested that the immaturity of catecholamine metabolism may correlate to the poor prognosis and that "dopaminergic neuroblastoma" may be a clinical subentity of poor prognostic neuroblastoma.
 ...
PMID:Dopaminergic neuroblastoma as a poor prognostic subgroup. 338 88

Tumor samples from 58 patients diagnosed and treated for neuroblastoma or ganglioneuroblastoma in a single institution from 1967 to 1981 were included in a study of prognostic factors. Histopathology, certain immunohistochemical markers, and DNA ploidy were evaluated along with clinical variables such as tumor stage, primary site, and patient's age at diagnosis. Children under 1.5 years of age at diagnosis had a much better prognosis than did older ones, and this variable was the best prognostic indicator. Tumor Stages I to II and IVS, primary tumor site above the diaphragm, and tumor differentiation were also related to a better prognosis. The Shimada classification was of no additional prognostic value in our study. Neither was the immunohistochemical marker pattern, but it was sometimes helpful in establishing the tumor diagnosis. Tumor-cell ploidy, however, seemed to afford additional prognostic information because the 11 patients with aneuploid tumors under the age of 1.5 years at diagnosis all survived in contrast to only five of nine patients with diploid tumors in the same age group. It is possible that this was due to a better response to treatment in the aneuploid group. Our results suggest that patients with diploid neuroblastomas of undifferentiated histology and those over the age of 1.5 years at diagnosis might be selected for more intense treatment.
 ...
PMID:Prognostic factors in neuroblastoma. Clinical, histopathologic, and immunohistochemical features and DNA ploidy in relation to prognosis. 339 59

Six new cell lines have been established from human neuroblastomas. Cell line SMS-KAN, from primary tumor before therapy, and line SMS-KANR, from bone marrow after chemotherapy and radiotherapy, were established from the same patient. Cell lines SMS-KCN (from primary tumor before any therapy) and SMS-KCNR (from bone marrow after chemotherapy) were established from another patient. Two other lines (SMS-MSN and SMS-SAN) were established from different patients before any therapy was given. Cell lines established from recurrent disease after chemotherapy (SMS-KANR and SMS-KCNR) had significantly shorter doubling times and increased plating efficiencies compared to those of cell lines derived from the same patient before chemotherapy (SMS-KAN and SMS-KCN). All cell lines contained tyrosine hydroxylase, aromatic L-amino acid decarboxylase, and dopamine-beta-hydroxylase. Measurable amounts of choline acetyltransferase were also detected in SMS-KAN and SMS-KANR. Karyotype analysis showed all cell lines except SMS-MSN to be pseudodiploid with modal numbers of 46 and deletions of the short arm of chromosome 1; SMS-MSN had a modal number of 57-58 chromosomes. All cell lines had double-minute chromosomes, except SMS-KANR, which had abnormally banding regions. These new cell lines provide in vitro models of neuroblastoma suitable for the study of differences in neuroblastoma cell populations before chemotherapy as compared to the cell populations that proliferate after therapy.
 ...
PMID:Characterization of human neuroblastoma cell lines established before and after therapy. 345 56

This paper reports late effects and health status of 198 children who had cancer or leukemia diagnosed under 2 years of age and their therapies electively withdrawn. This series (92 neuroblastoma [NBL], 57 Wilms' tumor [WT], 46 acute lymphoblastic leukemia [ALL], and 3 non-Hodgkin's lymphoma) was followed for 1-12 years after discontinuation of therapy. Thirty-three children were diagnosed before 1973, 92 between 1973 and 1977, and 73 after 1977 in 16 Italian Pediatric Oncology Centers. As of December 1983, 176 children were reported to be alive and without evidence of primary cancer by physicians responsible for their care. One child died from a second primary tumor, two from late recurrences of the primary cancer, and three from other causes; eight were alive with evidence of primary cancer; and eight were lost to follow-up. Kyphoscoliosis was found in 22 children and other musculoskeletal anomalies in 8. Neurological sequelae were observed in 8 out 35 children with ALL treated with radiotherapy (RT) and intrathecal methotrexate. All but one were in continuous complete remission when they developed seizures (three cases), leukoencephalopathy (three cases), or intracerebral calcifications (two cases). One child had cardiomyopathy and subsequently died from cardiac failure: he had received doxorubicin (400 mg/m2) and mediastinal RT (13 Gy) for NBL. Growth impairments were observed in children with NBL and WT.
 ...
PMID:Health status of young children with cancer following discontinuation of therapy. 347 May 93

Children with neuroblastoma presenting with opsomyoclonus are a diagnostic dilemma; they may not have a palpable mass or elevated urine catecholamines and the neurologic manifestation often precedes the discovery of a primary tumor by several months. The delay in diagnosis probably reflects the difficulty in detecting small lesions. Even with high-resolution computed tomography (CT), difficulty was recently experienced in identifying small retroperitoneal lesions in three patients who presented with opsomyoclonus. Calcifications in these small neoplasms resembled contrast material within adjacent small-bowel loops that were not discernible until additional, unenhanced scans were obtained. It is recommended that the initial CT evaluation in patients presenting with opsomyoclonus be performed without bowel or intravenous contrast enhancement.
 ...
PMID:CT scanning in patients with opsomyoclonus: importance of nonenhanced scan. 348 51

Twenty-six studies by meta-(131I)-iodobenzylguanidine scintigraphy (131I-MIBG), 26 studies by 67Ga-citrate and 33 99mTc-hydorxymethylene diphosphate (99mTc-HMDP) scintigraphic studies were performed for 10 patients with abdominal neuroblastoma. Comparing the 131I-MIBG images obtained at 24, 48 and 72 h, the 48-h image was the most distinctive for the tumor. Intrabdominal primary lesions, which ranged from bean to fist-size, were visualized in 7/7 cases (100%) by 131I-MIBG, 4/7 cases (57%) by 67Ga-citrate and 4/8 cases (50%) by 99mTc-HMDP before surgery and at diagnosis. In serial follow-up of these patients after starting chemotherapy, 131I-MIGB detected 100% of regressing primary tumors. Studies of 5 postoperative patients showed negative images for the primary tumor in all 3 scintigraphies except one in whom 131I-MIBG was positive, but not 67Ga-citrate or 99mTc-HMDP, for an unresectable residual tumor. 131I-MIBG also detected metastatic lesions not predicted by 67Ga-citrate or 99mTc-HMDP and reflected tumor progression more sensitively than known tumor markers such as urinary vanillylmandelic acid (VMA), homovanillic acid (HVA), serum neuron-specific enolase (NSE) and ferritin. These findings indicate that the 48 hr 131I-MIBG scintigraphy is superior to 67Ga-citrate or 99mTc-HMDP images and to other biochemical markers in monitoring the effect of treatment on neuroblastoma.
 ...
PMID:131I-meta-iodobenzylguanidine scintigraphy in patients with neuroblastoma. 360 63


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>