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 authors would like to recount and discuss the case of a 14 months old child which presented a dumbbell dorsal lipoma, purely extradural with no congenital anomalies. The warning signal was the discovery of paraparesis with the regression of walking ability. The hypothesis of medullary compression by a dumbbell dorsal tumor was quickly dismissed with the discovery of a mediastinal mass on the pulmonary X-Ray. A diagnosis of the pre-operational neuroblastoma was carried out because of detection of dorsal epidural medullary compression revealed by the myelography. The testing was completed with a vertebral computed tomography, which clearly showed the extent of medullar compression, and the mediastinal tumoral development under the paravertebral muscular masses. The child was operated on at 2 fronts, vertebrally and thoraxically for an encapsulated, perfectly separable and non hemorrhaging tumor. The anatomopathological examination confirmed the diagnosis of the lipoma. It is in the resumption of the computed tomographic examination that the tumoral density measures will confirm fatty tissue with numbers of density--50. In light of the literature, we are discussing the appearance of the lipoma, entirely exceptional because of its dorsal location, its extradural and certainly its dumbbell position, with large thoraxic development. It is necessary to stress the importance of the meticulous neurological examination of the child, the diagnostical necessity of the CT scan and the importance of the avoidance of the creation of an instable vertebral lesion at the time of laminotomy. In this case only the early double surgical operation permitted the recovery of the child.
 ...
PMID:[Dorsal extradural hourglass lipoma in children]. 673 27

Epidural neuroblastoma xenografts in nude rats causing paraparesis were treated with intravenous injection of an anti-GD2 monoclonal antibody 3F8. Metastatic or primary epidural tumors in humans cause rapid neurologic compromise. Treatment is often unsatisfactory. An animal model was established to study antibody targeted therapy of epidural tumor. Human neuroblastoma was xenotransplanted into the thoracic epidural space of nude rats. When paraparesis developed, animals were treated intravenously with an anti-GD2 monoclonal antibody, 3F8, either alone or radiolabeled with 131Iodine. Improvement in neurologic function occurred in 2 of 20 (10%) animals receiving no treatment or control antibody, 14 of 17 (82%) animals receiving 3F8 alone and all 9 animals receiving 131I-3F8 (p < 0.0001 for 3F8 or 131I-3F8 vs. control). Six animals treated with 3F8 alone recovered normal neurologic function and remained well until sacrifice 10 days later. Four animals treated with 3F8 alone had no tumor evident on pathologic examination. The percent injected dose of 131I-3F8/g tumor in 5 samples ranged from 0.73% to 3.8%. These observations demonstrate that neoplastic epidural compression of the spinal cord in the rat can be treated successfully with intravenous unmodified monoclonal antibody and that signs of neurologic dysfunction can be reversed. The potential of this approach in treating patients with epidural tumors and other neoplasms, especially those that are not sensitive to chemotherapy or radiotherapy, deserves to be explored.
 ...
PMID:Treatment of spinal epidural neuroblastoma xenografts in rats using anti-GD2 monoclonal antibody 3F8. 836 Jul 9

Neuroblastoma is an embryonic tumor of the sympathetic nervous system originating from neural crest cell remnants, which may be found in the adrenal glands or sympathetic chain. We report a case of a three-year-old girl who presented with flaccid paraparesis, and acute respiratory distress that required mechanical ventilation after a multiple-day history of gradual-onset dyspnea and right upper-lung opacity that was initially misdiagnosed as pneumonia. Chest and spinal magnetic resonance imaging (MRI) revealed a tumor in the posterior mediastinum with intra-spinal canal extension. The patient underwent thoracotomy and surgical en bloc resection with spinal canal decompression. Histopathology revealed neuroblastoma. The postoperative period was uneventful. The patient was administered a course of chemotherapy but died due to advanced stage tumor, severe pancytopenia, and septicemia with renal failure. The aim of this report is to consider posterior mediastinal neuroblastoma in the differential diagnosis of dyspnea and acute lower limb weakness in young children.
 ...
PMID:Posterior mediastinal neuroblastoma masked as flaccid paraparesis in a 3 year child. 3187 13