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 experience of one regional thoracic surgical unit in managing intrathoracic neural tumours over a 25-year period is presented. Neural tumour was diagnosed in 55 patients, of whom 41 were asymptomatic. In 11 patients complete resection was not achieved--the reasons for this and its effect on the outcome of the patient are discussed. There were 52 posterior mediastinal and three lateral chest wall tumours. The pathological distribution was as follows--benign nerve sheath tumours (neurofibroma, neurilemoma) 39, ganglioneuroma 13, and neuroblastoma 3. One neurofibroma recurred as a neurosarcoma six years after its apparently complete resection and was removed by an extensive resection at reoperation. One neuroblastoma recurred within the spinal canal four years after incomplete excision at thoracotomy--this patient died subsequently of widespread metastatic neuroblastoma. No other tumour is known to have recurred.
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PMID:Intrathoracic neural tumours. 21 May 31

Application of computed tomography (CT) to neck masses has received little attention. The authors reviewed 10 cervical masses studied with CT as well as conventional imaging modalities. CT was extremely useful in defining both the osseous and soft-tissue extent of the lesion. In several instances, CT was able to show the relationship of the tumor to the spinal canal. When combined with angiography, CT demonstrated the relationship of the major cervical vascular channels to the lesion. Pathological conditions included neurofibroma, chordoma, branchial cleft cyst, neuroblastoma, lymphoma, neurilemmoma, and metastatic carcinoma.
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PMID:The role of computed tomography in the evaluation of neck masses. 47 83

A group of bioptically examined neuroectodermal tumours of the skin and soft tissues consisting, in particular, of 170 Schwannomas and 350 tumours has been reevaluated in a retrospective study. The following common classification of these tumours has been recommended: I. Tumours of Schwann's cells: 1. neurilemmoma [A and B], 2. neurofibroma [with the following variants v. Recklinghausen's type, plexiform, pigmented, Paccinian, with Meissner-Wagner's bodies and meningiomatous], 3. amputation neuroma, 4. neurosarcoma, 5. others. II. Melanogenic tumours: A. pigmented naevi (junction, mixed, intradermal, epithelioid, clear cell, halo, neurocutaneous, fibrous, blue, proliferating blue, melanotic progonoma, others). -B. praecancerous melanosis. -C. malignant melanoblastoma (common type, from praecancerosis). III. Tumours of ganglion cells: 1. ganglioneuroma, 2. neuroblastoma, 3. paragangliomas (with granules, without granules, alveolar soft part sarcoma).
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PMID:[Neuroectodermal tumors of the skin (a normative study)]. 59 24

160 tumors of neural origin occurring in the thorax were analyzed. The major histological features of schwannoma, neurofibroma, neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and paraganglioma are described. Radiological analysis emphasized shape and location. Calcification was relatively uncommon but may be specific. The comparatively low figures on incidence of rib and vertebral abnormalities might be increased by special studies, including vertebral tomography. Evidence of local spread such as pleura-based nodules and pleural effusion constitutes evidence of malignancy. Age may be the most important clinical parameter for distinguishing between histological types.
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PMID:Neural tumors of the thorax: subject review from the AFIP. 61 41

This classification is arranged in two parts in order to take into account the different origins, structures, and functions of the cortex and medulla. The tabular classification is a simplified version of that suggested for adrenal tumours in man, and includes cortical adenoma and carcinoma, phaeochromocytoma, chemodectoma, neurofibroma, ganglioneuroma and ganglioneuroblastoma, and neuroblastoma. A detailed functional classification is not given, since the hormonal activity of many adrenal tumours in animals is less well known than it is in man. Of the tumour-like lesions listed, cortical hyperplasia is particularly important in several species.
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PMID:Tumours of the adrenal gland and paraganglia. 108 53

We describe the histological, immunohistochemical, and ultrastructural findings of a cutaneous tumour composed of ganglion cells, without any other proliferating component. As ganglion cells are not normal components of the skin, we propose the term "ganglion cell choristoma" for this lesion. The differential diagnosis of related lesions such as cutaneous ganglioneuroma, well-differentiated metastases from neuroblastoma, autonomic ganglia entrapped by neurofibroma, and reactive processes, and the possible histogenesis of ganglion cell choristoma are discussed.
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PMID:Cutaneous ganglion cell choristoma. Report of a case. 177 58

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

Two kinds of novel neural trophic factors were currently detected in von Recklinghausen neurofibroma (NF1) extracts. One of the two was a growth factor, neuroblastoma growth factor (Mr less than 5 kDa), which promotes the proliferation of human neuroblastoma cell and survival and neurite-extension of rat cortical neurons, but differently from nerve growth factor (NGF) or NGF-like factors. The other one was a glial growth inhibitor (Mr = 100 kDa), which suppresses the growth of glioma cell lines, astrocytoma, glioblastoma, oligodendroglioma and Schwannoma. These factors do not appear to be previously identified cytokines or growth factors such as interleukins, granulocyte colony-stimulating factor, NGF and fibroblast growth factor. There was also detectable ciliary neurotrophic factor-like activity in the extracts. The primary cause of high contents of these factors in NF1 is not known, but may relate to fundamental mechanisms controlling growth and differentiation of neurons and glias during development of nervous system.
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PMID:von Recklinghausen neurofibroma produces neuronal and glial growth-modulating factors. 193 68

Neurofibroma and neuroblastoma both arise from the neural crest, and there has long been speculation regarding a pathogenetic relationship between them. Clinical characteristics do not necessarily distinguish these tumors, therefore the diagnosis of neuroblastoma should be considered in all children with neurofibromatosis 1 (NF-1) who have a rapidly growing or inaccessible mass. A careful physical examination, imaging studies, and urinary catecholamine measurement are indicated. In a child with NF-1 and malignancy, direct tissue examination may be necessary to differentiate malignant from nonmalignant tumor and guide therapy. Furthermore, with the significantly increased risk of certain types of childhood cancer in these patients, we recommend evaluation for this common heritable condition in all patients with malignancy.
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PMID:Neurofibromatosis 1: recognition and management of associated neuroblastoma. 212 42

Identification of growth factors and receptors in mesenchymal tumors may be crucial to understanding of growth regulation in sarcomas. During an immunohistochemical study of the expression of growth factors and receptors in human soft tissue tumors (STT), only 1 antisera capable of working in paraffin-embedded tissue was noted. A detailed study of 141 STT was undertaken to determine the frequency of expression of nerve growth factor receptor (NGF-R), its specificity and sensitivity for neural tumors, and the effect of fixation on detection. In normal mesenchymal tissue, only nerve sheath and perivascular staining was seen. No immunoreactivity was seen in many tumors including rhabdomyosarcoma, angiosarcoma, liposarcoma, Ewing's sarcoma, and alveolar soft part sarcoma. Less than 15% of tumors of smooth muscle, fibrous, or fibrohistiocytic origin showed immunoreactivity, usually focal. In contrast, a high frequency of immunoreactivity was noted in tumors of neural origin (74%). This included granular cell tumors (100%), Schwannoma/neurofibroma (91%), malignant Schwannoma (78%), neuroblastoma/neuroepithelioma (60%), and paraganglioma (57%). A high rate of reactivity was also seen in synovial sarcomas (80%), undifferentiated sarcomas (60%), and hemangiopericytomas (43%), suggesting a potential relationship to the neural phenotype. Among the neural tumors, Bouin's fixation was superior to formalin, suggesting that immunoreactivity for NGF-R is affected by fixation. This antibody may be a useful adjunct marker diagnostically.
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PMID:Expression of nerve growth factor receptor in paraffin-embedded soft tissue tumors. 245 20


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