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

A case of congenital neuroblastoma presenting with paraplegia in a newborn baby is described. The tumor was removed and chemotherapy was given. The child is now 2 years old, without relapse, but still suffers from flaccid paralysis and anaesthesia of both legs.
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PMID:Congenital neuroblastoma with paraplegia. Case report. 127 Mar 24

From 1970 to 1989, 121 children with mediastinal masses of various sorts were seen in the Department of Pathology, Royal Children's Hospital, Melbourne. The series is considered representative of the true incidence of these conditions in the state of Victoria, which had an average paediatric population during the time of this series of 900,000 children. The commonest cause of a mediastinal mass was NHL (36 cases). This was followed by HD (24 cases), then neuroblastoma and ganglioneuroma (16 and 9 cases respectively), duplication cysts (10 cases), teratomas (7 cases), neurofibroma (4 cases) and lymphangioma (3 cases). A great variety of rare conditions made up the remainder of the series and included mediastinal abscess, thymic cyst, pericardial cyst, accessory lobe of lung, plasma cell granuloma, fibromatosis, paravertebral Ewing's tumour, carcinoid tumour and neurofibrosarcoma. Presentation of the children with NHL was often acute with respiratory distress, while the child with HD was usually older and symptoms were more often systemic than local. The surgeon's role in diagnosis of these most frequently encountered mediastinal masses can be crucial and biopsy when indicated must be carried out with great care to produce material that is adequate for diagnosis and for the performance of cell marker studies and chromosome analysis. Neuroblastoma (NBL) and ganglioneuroma (GN) together were the third largest group. Children with neuroblastoma were usually young; 15 of the 18 cases were less than 2 years old. One-third of the infants with neuroblastoma presented with paraplegia and one-third with respiratory symptoms including wheeze, stridor and respiratory difficulty. Three children had Horner's syndrome. Prognosis of children with thoracic neuroblastoma is very good and contrasts with the poor outlook for those with abdominal neuroblastoma. Stage at presentation is probably the most important single prognostic variable. Ganglioneuroma presents at a later age than neuroblastoma and symptoms may be present for a long time or may be completely absent. Catecholamines, usually raised in neuroblastoma, are mostly normal in ganglioneuroma. Duplication cysts were the next most frequent group. Symptoms can often be acute and life threatening, although in three of our ten cases the cyst was an incidental finding on chest X-ray. However, only three of our patients had a normal respiratory examination. Teratomas were usually large and more often benign than malignant. Excision is the mandatory treatment and is usually curative. Although teratomas in young infants are often cellular and composed of many immature tissue types, their behaviour is benign.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Mediastinal masses in childhood: a review from a paediatric pathologist's point of view. 190 92

A girl presented with paraplegia at birth. A magnetic resonance scan showed an abdominal neuroblastoma with intraspinal extension. Treatment by chemotherapy did not produce any improvement in the neurological signs. Review of previously reported cases suggests the outlook for neurological recovery is poor regardless of treatment.
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PMID:Congenital neuroblastoma presenting with paraplegia. 195 14

Adult neuroblastoma (ANB) is an uncommon malignancy, there being only 42 reported cases in the world literature. The purpose of this report is to present a case of ANB with spinal cord compression by an intraspinal component, and review methods of diagnosis and treatment. NB is one of the most frequent childhood malignancies (90% of the patients are less than 10 years old) and both pathologists and clinicians tend to exclude it from differential diagnosis when the patient is an adult. When the tumour is located in the paraspinal region, local extension through the intervertebral foramen into the spinal canal can occur in a dumbbell fashion, a pattern of growth first described by Weber in 1856.
Paraplegia 1989 Oct
PMID:Neuroblastoma in an adult causing spinal cord compression: report of a case and review of the literature. 260 90

The clinical presentation, diagnosis and treatment of a series of 18 cases of neuroblastoma is reviewed. About half the patients were below the age of 2 years. Fever, loss of weight and appetite appeared to be the common manifestations, underscoring the vague and non-specific symptomatology of such a grave childhood disorder. Unusual presentations included paraplegia, chronic diarrhoea, lower limb edema and respiratory distress. Management consisted of radical surgery with adjunctive chemotherapy where indicated. It is emphasised that in resectable lesions every attempt must be made to remove tumour involved tissue as thoroughly as possible. With this approach 50% of patients followed up to 4 years have survived. Comparison of characteristics of survivors against non-survivors showed that the only significant difference was in the state of the disease--survivors having generally earlier state tumours.
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PMID:Experiences in the treatment of neuroblastoma. 319 Jan 52

Autonomic dysreflexia and catecholamine secreting tumor, each of which causes paroxysmal hypertension, coexisted in a young man. Two years after neuroblastoma was diagnosed, he developed T4 incomplete paraplegia due to metastases to the spine at T5 and L3 levels. Shortly after the onset of paraplegia, paroxysmal hypertension developed. The hypertension was controlled adequately by good bowel and bladder management and oral clonidine. The paroxysmal hypertension is believed to have resulted from the synergistic effect of the high levels of circulating catecholamines from the tumor and the disruption of autonomic pathways.
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PMID:Autonomic dysreflexia in a paraplegic man with catecholamine-secreting neuroblastoma. 374 Oct 83

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

Lesions from the SC region of children examined histologically at the RAHC were: 1. Malformations almost always associated with spina bifida aperta or occulta: 183 myelomeningocele (MM), 32 meningocele (M), 35 lipoMM and lipoma, 19 dermoid cyst, six occult meningocele, two Pacinian hamartoma, one short filum, four hindgut cysts or sinuses, two tailgut cysts, and two epithelial heterotopia. 2. Neoplasms, usually without spina bifida: 56 teratomas (11 malignant), five ependymomas (two purely subcutaneous), and 14 miscellaneous primary malignancies, (most neuroblastoma and rhabdomyosarcoma). Distinction between MM with glial tissue and M without glial tissue is important as M had a much better prognosis, less than a third developing hydrocephalus, and 77% walking unaided. Of those with glial tissue, the eight without Arnold-Chiari malformation were myelocystocele associated with cloacal exstrophy (six), caudal regression syndrome (one), and microcephaly (one). Postsacral glial tissue without paraplegia may occur with a subcutaneous vestige of filum terminale, or with herniation of the nonfunctioning half of a diplomyelia. Of postsacral "lipomas" and dermoids, 70% had an intraspinal connection through an occult spina bifida. This posterior vertebral defect is easily overlooked as the arches normally may not ossify until after 6 years. Therefore, the pathologist receiving a postsacral specimen may wish to alert the clinician to the high incidence of late effects from an occult intraspinal component or tethering of the spinal cord. Transsacral hindgut herniations and cysts probably result from ectoendodermal adhesions. Presacral multicystic malformations with mixed squamous and mucus cell lining are probably tailgut remnants or anorectal duplications, and may be mistaken for dermoid or teratoma. In SC teratoma in infants, contrary to some reports on ovarian teratoma in adults, immature tissues do not indicate a worse prognosis. Malignancy is virtually confined to teratomas including a carcinomatous or "yolk sac" component. It is more common in predominantly presacral examples and rare before the age of 4 months. SC ependymoma differs from ependymoma elsewhere in that it may be primary outside the craniospinal cavity (presacral or postsacral), may have a myxopapillary pattern special to the region, and although low-grade and slow growing, is more likely to metastasize beyond the central nervous system. Postsacral examples arise from vestiges of the filum terminale which are normal in the subcutis there. Combinations of all these lesions occur with vertebral defects and with each other.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Sacrococcygeal developmental abnormalities and tumors in children. 636 33

Spinal cord compression is a rare but serious complication of malignant diseases in children. Epidural cord compression was noted in 81 patients within the past 17 years at this center. The complication developed at different times during the course of the primary disease. For 29 of our patients, cord dysfunction was one of the initial signs of cancer--Ewing sarcoma, neuroblastoma, Hodgkin disease, and malignant lymphoma. By contrast, for most of the patients with osteosarcoma and rhabdomyosarcoma, it appeared later in their clinical course. The treatment outcome of patients who were paraplegia with complete loss of sensory function for greater than or equal to 48 hours was poor. Only four of 22 in this group became ambulatory. Ten patients with osteosarcoma did not undergo laminectomies because they all had multiple metastases and terminal disease. Paraplegia developed in all ten. There was no difference in ambulatory rates among other patients, with or without laminectomies.
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PMID:Metastatic epidural tumors in children. 695 58

Neuroblastomas appear to be one of the most frequent congenital solid tumors. Nevertheless, intraspinal congenital neuroblastomas are uncommon. The authors report seven cases of congenital intraspinal neuroblastomas operated on in the "Service de Neurochirurgie des Enfants-Malades" from 1970 to 1980. Their study and that of 16 cases published in the literature allows to stress several points (7, 12). The dumb-bell intraspinal neuroblastomas present a large extension. They usually determine a severe and definitive paraplegia. They probably have a long prenatal evolution. The tumoral prognosis of these congenital neuroblastomas is good. Success should be expected in nearly 80% of the cases. Surgery, in two steps, intra and extra spinal, remains compulsory even though the neurological prognosis is poor. It is useless to operate upon the neuroblastomas as emergencies, when neurological involvement is total at birth. The complementary treatment, X ray-therapy or chemotherapy is not without any danger on newborns. It necessary is not proved.
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PMID:[Congenital intraspinal neuroblastomas. A propos of 7 cases treated surgically]. 717 10


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