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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The roentgenologic diagnosis and differentiation of mediastinal neurogenic tumors are possible on the chest roentgenogram as a rule. The soft tissue mass may be ill-defined and the tumor "ghost-like" in the case of primary neuroblastoma, but it is usually obvious in ganglioneuroma and metastatic disease. The presence of calcifications differentiates neurogenic tumors from other posterior mediastinal tumors of childhood. They are common in primary and rare in secondary disease. Rib erosions and displacement are striking in neuroblastoma (after a few months of age), more subtle in ganglioneuroma, and absent with secondary involvement. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis and treatment were delayed, as the adjacent rib changes were not appreciated for some time. "Dumbbell" shaped tumors are usually associated with vertebral changes and myelography is indicated even in the absence of neurologic deficit. Thoracic deformity and disability subsequent to laminectomy, radiation therapy, or both, are present in all survivors.
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PMID:Mediastinal neuroblastoma and ganglioneuroma. The differentiation between primary and secondary involvement on the chest roentgenogram. 120 Feb 37

Neuroblastoma, Hirschsprung's disease, and central hypoventilation (Ondine's curse) are considered aberrations of neural crest cell growth, migration, or differentiation, and as such are considered to be under the general heading of neurocristopathy. Their combined occurrence in a newborn infant presenting with total colonic aganglionosis, central hypoventilation, and multifocal neuroblastoma had not been reported previously. A 2.3-kg white full-term girl required endotracheal intubation because of persistent apnea in the first hours of life. She had progressive abdominal distension and failure to pass meconium; a barium enema was performed, which showed microcolon with meconium pellets at the distal ileum. During laparotomy the distal ileum was found to be obstructed with inspissated meconium; an ileostomy and appendectomy were performed. The resected specimens were aganglionic. An additional 20 cm of aganglionic ileum was removed, and a normally innervated ileostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an extensive apnea workup, including electroencephalogram, magnetic resonance imaging (MRI), bronchoscopy, and pH probe study, were normal. Sleep studies showed congenital central hypoventilation syndrome, and the patient underwent a tracheostomy. At 3 months, an abdominal ultrasound examination performed within a septic workup showed a right suprarenal mass extending across the midline. Thoracic and abdominal MRI scans showed large bilateral adrenal and posterior mediastinal masses. The serum catecholamines and ferritin level were markedly elevated, suggestive of neuroblastoma. In light of the child's multiple problems, the family chose to forgo further workup (including a tissue biopsy) and therapy. In the following 2 months her tumor load rapidly progressed, and she died of respiratory insufficiency.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The complete spectrum of neurocristopathy in an infant with congenital hypoventilation, Hirschsprung's disease, and neuroblastoma. 747 88

Thoracic (mediastinal) neuroblastomas (NB) have been reported to differ from abdominal (suprarenal and retroperitoneal) NB and to be associated with better prognosis. The comparison between them is rarely published. In this retrospective study, the characteristics of thoracic NB (17 cases) are investigated and compared with abdominal NB (51 cases). Regarding the diagnosis, thoracic NB presented in lower clinical stages I and II in 35.3% of cases, compared to 11.7% of abdominal NB in stages I and II (p<0.001). The disease was initially diagnosed at less than one year of age in 7/17 (41.2%) of thoracic NB and in 12/51 (23.5%) in abdominal cases (p<0.001). The median age at the time of initial diagnosis was 15.3 months for thoracic NB and 27.6 months for abdominal neuroblastoma (p<0.05). The cases with an elevated vanillylmandelic acid (VMA) and homovanyillic acid (HVA) excretion were 9/17 (52.9%) in the mediastinal NB, and 43/51 (84.3%) in the abdominal NB, respectively (p<0.05). The quantitative values of tumour markers were significantly lower in thoracic NB (0.85 vs. 2.14, p<0.001). Regarding surgery, complete tumour resection was achieved in 15/17 thoracic NB (88.2%) compared to 36/51 (70.6%) radicality in abdominal NB. Surgical complications developed in 5/17 thoracic procedures (29.4%) without a lethal outcome. The mean tumour mass of thoracic NB was 56.5 g vs. 106.3 g of abdominal neuroblastoma (p<0.001). The incidence of ganglioneuroblastoma in mediastinal tumours was 3/17 (17.6%) compared to 8/51 (15.7%) in abdominal NB (non significant). A favorable histology based on Shimada classification was found in 37% of the mediastinal neuroblastoma cases and in 22% in the abdominal NB cases (p<0.05). Regarding the biological properties, genetic malformations associated with NB were identified in 2 thoracic cases (1p deletion and polyploidy). Genetic changes were identified in 12 cases of abdominal NB (1p deletion in 4 cases, DNA ploidy in 6 cases, N-myc amplification in 1 case). One additional abdominal NB had 1p deletion, DNA ploidy and N-myc amplification. This study supports results of other investigations that thoracic NB differs significantly in many aspects from abdominal NB. Important differences in favorable histology and biological properties of thoracic NB have changed the concept of surgical treatment, although unnecessary attempts of surgical radicality still lead to serious complications. Complete excision remains the mainstay of therapy of localised thoracic NB, while in most abdominal tumours the aim of an initial operation should be sampling of tumour tissue for histology and molecular biological examination, with complete excision of the mass as the second priority.
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PMID:[The characteristics of mediastinal neuroblastoma and perspectives on surgical excision]. 1530 5

The purpose of this report is to describe deployment of the Relay NBS Thoracic Stent Graft with the Plus Delivery System (Bolton Medical, Sunrise, FL) in a flexible resin arch model with a 15-mm radius curve as well as our preliminary clinical results. The Relay NBS graft with the Plus Delivery System was evaluated by way of bench testing, which was performed with stent grafts with diameters ranging from 24 to 46 mm and lengths ranging from 100 to 250 mm in flexible resin arch models with a 15-mm arch radius of curvature. The deployment sequence was analyzed. The Relay NBS graft with the Plus Delivery System was deployed in two patients, respectively, having a 6.5-cm penetrating aortic ulcer of the proximal third of the descending thoracic aorta and a DeBakey type-I aortic dissection with chronic false lumen dilatation after surgery due to an entry site at the distal thoracic aorta. Bench tests showed proper conformation and apposition of the Relay NBS graft with the Plus Delivery System in the flexible resin model. This stent graft was deployed successfully into the two patients with a correct orientation of the first stent and without early or late complications. The Relay NBS graft with the Plus Delivery System ensures an optimal conformation and apposition of the first stent in the aortic arch with a small radius of curvature.
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PMID:Relay NBS graft with the plus delivery system to improve deployment in aortic Arch with small radius curve. 2039 87