Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The indication for radiotherapy in neuroblastoma depends on the stage of the disease; the irradiation dosage depends on the age of the child. The tumour is radiosensitive, but tends to recur if the irradiation dosage is inadequate. Irradiation of the tumour may induce coversion to a more differentiated tumor pattern. Radiotherapy can reduce the tumour volume, thereby rendering previously inoperable tumours resectable. Since 1959 11 out of 16 children with neuroblastoma undergoing treatment at the Paediatric Department of the University of Vienna were subjected to radiotherapy. Out of 9 children with suprarenal origin of the tumour only one child is still alive (survival time to date-26 months since diagnosis). Out of 7 children with extraadrenal tumour localization 70% have survived for more than 2 years. The side effects of radiotherapy survived for more than 2 years. The side effects of radiotherapy were: 1 case of quadripelgia, 1 case of symmetric growth delay of the pelvis and 1 case of scoliosis.
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PMID:[Radiotherapy of neuroblastoma (author's transl)]. 26 32

Thirteen children with localized (Evans stage I or II) thoracic primary neuroblastoma were divided into two groups according to the type of therapy administered, in order to compare the therapeutic efficacy and morbidity of excisional surgery followed by either irradiation alone or irradiation plus chemotherapy (group A) with similar surgery alone (group B). Group A consisted of 6 children (mean age 1 year, 2 months). Complete surgical excision was accomplished in 2 patients, while 4 had microscopic residual. All 6 patients are free of disease at 26--76 months (mean 47 months), including 2 who had recurrent tumor and received additional therapy. Two have developed congestive heart failure and one severe scoliosis secondary to irradiation. Of the 7 children in group B (mean age 2 years, 2 months), 3 had microscopic residual tumor and 2 had adjacent lymph node involvement. After 12--47 months (mean 23 months), no recurrence or surgery-related morbidity has been observed. From these limited data it appears that surgery alone may provide adequate therapy for localized thoracic neuroblastoma and obviate the morbidity associated with multimodal therapy.
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PMID:Management of localized thoracic neuroblastoma. 50 72

In order to investigate the number, types, severity and the various treatments of spinal deformity among children under 10 years old, a retrospective survey of registered patients in the scoliosis clinic at the National Taiwan University Hospital was performed. Between August 1982 and December 1988, there were 41 children who had scoliosis with a Cobb angle larger than 10 degrees and the onset was before 10 years of age. This number accounted for 3.8% of all scoliotic patients during the same time period. Of these children, 19 had idiopathic scoliosis (46.3%), 7 with infantile and 12 with juvenile; the other 19 were due to congenital, and the remaining 3 were postradiation, a resection of Wilms' tumor in 2 and neuroblastoma in 1. In the congenital group, hemivertebra (13 patients) outnumbered other causes. Twenty three patients (56%) underwent surgical correction, the rest were either under regular observation (9 patients), bracing (7 patients) or electrical stimulation (2 patients). The average preoperative Cobb angle in the operated groups was much larger, being 67.6 degrees in the infantile; 52.4 degrees in the juvenile; 57 degrees in the congenital; and 62 degrees in the postradiation. For those without an operation, the angles were smaller than 30 degrees. The indications for surgery were that the curvature was in progression, which could not be controlled by conservative means, or that in some congenital cases, the curve had the potential tendency to exacerbate. From the present study, the percentage of scoliosis under 10 years of age was far less than the adolescent group in our clinic.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Spinal deformities among children under 10 years old: a clinical analysis of 41 cases. 198 37

One hundred forty-three patients who received radiation therapy for childhood tumors, and survived to the age of skeletal maturity, were studied by retrospective review of oncology records and roentgenograms. Diagnoses for the patients were the following: Hodgkin's lymphoma (44), Wilms's tumor (30), acute lymphocytic leukemia (26), non-Hodgkin's lymphoma (18), Ewing's sarcoma (nine), rhabdomyosarcoma (six), neuroblastoma (six), and others (four). Age at the follow-up examination averaged 18 years (range, 14-28 years). Average length of follow-up study was 9.9 years (range, two to 18 years). Asymmetry of the chest and ribs was seen in 51 (36%) of these children. Fifty (35%) had scoliosis; 14 had kyphosis. In two children, the scoliosis was treated with a brace, while one developed significant kyphosing scoliosis after laminectomy and had spinal fusion. Twenty-three (16%) patients complained of significant pain at the radiation sites. Twelve of the patients developed leg-length inequality; eight of those were symptomatic. Three patients developed second primary tumors. Currently, the incidence of significant skeletal sequelae is lower and the manifestations are less severe than reported in the years from 1940 to 1970. The reduction in skeletal complications may be attributed to shielding of growth centers, symmetric field selection, decreased total radiation doses, and sequence changes in chemotherapy.
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PMID:Skeletal sequelae of radiation therapy for malignant childhood tumors. 213 23

This paper is the report of three cases of spinal deformities connected to a paravertebral ganglioneuroma: The first case was discovered during the anterior approach of a thoracic scoliosis of more than 100 degrees at the age of twelve; the child had been treated before the age of one year for a thoracic neuroblastoma; eleven years after removal of the ganglioneuroma and fusion of the spinal curve, the evolution is satisfactory. The second case was similar, but the initial findings during infancy were not well known; the result is good two years after excision of the tumor and fusion of the spine. The third case is simply a progressive kyphosis after removal of a thoracic ganglioneuroma by laminectomy at the age of five years. The sister of this child suffered of a malignant thoracic neuroblastoma. Are pointed out here below the nature of these ganglioneuromas, non-secreting tumors from neuroectodermic origin, their rarity in relation with spinal deformities, the difficulties of their detection by modern imaging, and the requirement of a close survey of these patients and their family.
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PMID:[Ganglioneuroma and scoliosis. Report of 3 cases]. 227 49

We have evaluated the role of radiotherapy in providing local control of primary tumors and to palliate metastases from neuroblastoma (NB). Fifty-five children with histologically verified NB were evaluated and treated from 1967 to 1984. In univariate analysis, the actuarial survival of eight children with thoracic primaries (85%) was significantly better than the survival of 39 children with intra-abdominal primaries (35%, p = 0.0287). The survival of 28 children less than or equal to 18 months of age at diagnoses was 73%, whereas 27 children older than 18 months had a survival probability of 10% (p = 0.0001). The survival by Evans stage was: I 100% (2 patients), II 85% (7), III 60% (13), IV 4% (27) and IV-S 100% (6). According to the Pediatric Oncology Group (POG) staging system, the survival was: A 100% (3), B 66% (9), C 66% (9), D 23% (34). A multivariable analysis indicated that the Evans staging system was a more powerful indicator of prognosis than the POG system. The analysis also indicated that Evans stage and patient age were independent determinants of survival. The primary tumor site did not add significant prognostic information beyond these two factors. Children with Stage I disease were treated with surgery alone. Most children with Stages II and III disease were treated with surgery, irradiation, and Cyclophosphamide or Cyclophosphamide plus Vincristine. All seven patients with Stage II disease received post-operative irradiation to the primary tumor and were locally controlled with doses of 4.8 to 26.5 Gy. Eleven of the 13 patients with Stage III disease were irradiated post-operatively. Seven of these 11 patients were locally controlled with doses of 12 to 48.4 Gy. The four Stage III patients with in-field recurrences were older children with large radiotherapy fields and/or low doses administered. The Radiation Therapy Oncology Group pain score system was used to evaluate response of painful bony metastases to irradiation. A response was observed in 65% of the sites irradiated. A response was observed at 67% of the soft tissue metastases irradiated. Hepatomegaly causing respiratory embarrassment or inferior vena cava obstruction was treated with irradiation in seven patients. All patients responded with doses ranging from 5 to 24.4 Gy. Five of the 17 children who survived for more than 5 years following treatment had significant scoliosis or kyphosis secondary to vertebral body abnormalities in irradiated bones. All five children were irradiated at a young age with megavoltage equipment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Radiation therapy in the management of neuroblastoma: the Duke University Medical Center experience 1967-1984. 242 88

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.
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PMID:Chest wall resection and reconstruction for malignant conditions in childhood. 320 68

There were 150 children with neuroblastoma and 183 children with Wilms' tumor who received radiation therapy, orthovoltage up to 1967, and cobalt therapy thereafter. In all except two cases, radiation therapy crossed the midline. Of all of the children treated, 13 required spinal fusion. There were six with Wilms' tumor; three had kyphosis; and three had scoliosis. Nine fusions were done with three pseudoarthroses, one broken rod, and two hook pullouts. There were seven with neuroblastoma, of whom five had kyphosis and two had scoliosis. Nineteen spinal fusions were done on these seven patients for neuroblastoma; two developed paraplegia; seven had pseudoarthroses; two had broken rods; and one had infection. Complications were attributed to laminectomies (five of seven) and high orthovoltage dosage (3680 rad), causing bone death and destroyed bone growth, with resultant infantile-size spines. The authors continue to follow up postradiation patients and observe rapid deterioration of kyphosis and scoliosis during the adolescent growth spurt. The authors now recommend early and extensive combined two-stage, long anterior and posterior fusions for kyphosis of 35 degrees and over, and a posterior fusion with Harrington rod instrumentation with extensive bone grafting is done for early (35 degrees) pure scoliosis. Postoperative immobilization is much longer than for regular spine fusions--at least one year.
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PMID:Results of spinal fusion for radiation scoliosis. 630 Oct 77

The results of management of 14 patients with Stage IV-S neuroblastoma are reported. The treatment policy, although not consistent over this time span, in general used a combination of radiotherapy and chemotherapy or infrequently one modality alone. Twelve of 14 (86%) survived more than 6 years. One patient, with a solitary mediastinal primary tumor, died of rapidly progressive disease at three months. The other death occurred in a 4.5-year-old presenting with hepatomegaly at diagnosis followed by skeletal dissemination 2.5 years later. Thirteen of the patients were younger than 1 year of age. Of the 11 patients that received radiotherapy, 4 experienced mild asymptomatic scoliosis or kyphoscoliosis at 3 to 12 years after initial therapy. A review of the literature indicates that spontaneous regression in this tumor is very frequent; therefore, it is recommended that for the common presentation of massive hepatomegaly in an infant, close observation is warranted, unless life threatening complications occur. However, initial therapeutic intervention may be indicated in those patients with life threatening presentations. This data did not substantiate the necessity for complete surgical excision of the primary tumor, as has been suggested by others.
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PMID:Stage IV-S neuroblastoma. Results with definitive therapy. 670 96

Of seventy-four children who were treated at a mean age of seventeen months for neuroblastoma and survived more than five years, fifty-six (76 per cent) had spinal deformity due either to the disease or to the treatment after a mean follow-up of 12.9 years. Of these fifty-six, 50 per cent had post-radiation scoliosis (mean, 18 degrees; range, 5 to 79 degrees), and 16 per cent had post-radiation kyphosis, most frequently at the thoracolumbar junction (mean, 39 degrees; range, 13 to 61 degrees), at the time of follow-up. Two kyphotic thoracolumbar curve patterns were identified: (1) an angular kyphosis with a short radius of curvature and its apex at the twelfth thoracic and first lumbar vertebrae, and (2) a thoracic kyphosis with a long radius of curvature that extended into the lumbar spine. The post-radiation deformity--both the scoliosis and the kyphosis--progressed with growth, the scoliosis at a rate of 1 degree per year and the kyphosis at a rate of 3 degrees per year. Epidural spread of the neuroblastoma was associated with most of the cases of severe scoliosis and kyphosis. The deformity was due either to the laminectomy or to the paraplegia acting in conjunction with the radiation. Eighteen per cent of 419 children with this malignant disease survived more than five years, and of the survivors, 20 per cent had spinal deformity severe enough to warrant treatment. The factors associated with the development of spinal deformity in patient treated for neuroblastoma were: (1) orthovoltage radiation exceeding 3000 rads, (2) asymmetrical radiation of the spine, (3) thoracolumbar kyphosis, and (4) epidural spread of the tumor.
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PMID:Spinal deformity in children treated for neuroblastoma. 746 75


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