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)

Nine patients who had an osteosarcoma that had developed as a second malignant neoplasm in a previously irradiated site were managed at a major center for the treatment of tumors in children. The doses of radiation had averaged 4144 centigray (range, 2300 to 8000 centigray) and chemotherapy had been administered, when appropriate, for the primary malignant lesion (Ewing sarcoma, malignant fibrous histiocytoma, Hodgkin lymphoma, neuroblastoma, neurofibrosarcoma, rhabdomyosarcoma, and Wilms tumor). The interval between the initial treatment and the diagnosis of the secondary sarcoma averaged ten years and one month (range, five years and ten months to twenty-one years and nine months). Three patients were alive, two of them with active disease, at the time of writing. The other six had died within three years (average, fifteen months) after the second diagnosis. The prevalence of secondary osteosarcoma is increasing as the survival of children who have a malignant lesion increases. Plans for tumor therapy should take into account the risk of this complication, which is usually fatal.
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PMID:Osteosarcoma as a second malignant neoplasm in children. 152 94

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

We studied N-myc oncogene expression in 13 human neuroectodermal tumors and one teratoma by in situ hybridization. In four of six neuroblastomas, there was increased N-myc expression (15 to 49% of the cells). Many of the primitive neuroblastic cells had an increase of N-myc RNA not observed in the larger, more differentiated cells. Two neuroblastomas matured to ganglioneuromas; no biopsies performed during this progression expressed increased N-myc RNA. Three ganglioneuroblastomas, two tumors presenting as ganglioneuromas, a cerebral neuroectodermal tumor, a neurofibrosarcoma, and the teratoma did not have increased N-myc expression. The data obtained by in situ hybridization correlated well with data obtained by blot analysis. Neuroblastomas/ganglioneuroblastomas with a favorable course did not have appreciable elevation of N-myc expression over 10 to 77 mo of follow-up; thus N-myc may not be involved in the maintenance of the neoplastic state. However, such tumors with a fatal outcome 2 to 14 mo after diagnosis usually had elevated N-myc expression. These findings suggest a relationship between elevated levels of N-myc RNA and poor prognosis.
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PMID:Detection of N-myc oncogene expression in human neuroblastoma by in situ hybridization and blot analysis: relationship to clinical outcome. 242 91

The clinical and pathologic features of 43 dogs with neoplasia of the rostral cerebrum were reviewed. Primary brain tumors included meningioma, astrocytoma, neuroblastoma, oligodendroglioma, and ependymoma. Other tumors that secondarily affected these areas included solitary hemangiosarcoma, nasal neuroendocrine carcinoma, nasal cell adenocarcinoma, nasal squamous cell carcinoma, and nasal neurofibrosarcoma. Older dogs were usually affected (mean, 10 years), and meningioma was the most frequent tumor type. Thirty-one dogs (72% of total) had a late-onset (greater than 5 years of age) of either generalized seizures or behavior abnormalities, or both, with an initially normal neurologic examination. In these 31 dogs, a mean time of 78 days (range, 2 to 400 days) elapsed from the onset of seizures or behavior change to the detection of a persistently abnormal neurologic examination. In all 43 dogs, the time from the detection of neurologic deficits to death or euthanasia and necropsy ranged from 1 to 63 days (mean, 13 days). On the basis of this review, it appears that dogs with late-onset seizures or behavior change, or both, should be suspected of having tumors involving the rostral cerebrum, despite the absence of persistent neurologic deficits commonly associated with cerebral tumors. Further, the onset of abnormalities in the neurologic examination and the time of death seem to occur within predictable time periods.
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PMID:Clinical signs of tumors affecting the rostral cerebrum in 43 dogs. 322 60

The effect of cisplatin on the auditory nervous system was examined in five children using brainstem auditory evoked potentials (BAEPs). The cases comprized of two neuroblastoma, one yolk sac tumor one neurofibrosarcoma and one Hodgkin's disease. All patients except for one infant aged eight months showed a normal audiogram in the pre-treatment examination. Cisplatin was administered at a dose of 75-105 mg/m2 (body surface area). BAEP tests were performed after cisplatin treatment from two weeks to 20 months. The patients were tested at rest in bed using stimulation with a 3 KHz, 80-dBHL click at 150-msec intervals 2,000 times. Four cases showed an abnormal BAEP pattern in the post-treatment examination. Two of them showed not only delayed conduction velocity of the first wave but also auditory disturbance, but these findings were improved after discontinuation of drug administration. We concluded that cisplatin frequently affects the auditory nervous system, and that this disturbance might be transient in the early stage.
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PMID:[BAEPs of children with malignant tumor undergoing cisplatin treatment]. 395 84

The insulin receptor-related receptor (IRR) is a member of the insulin receptor family. So far no ligand has yet been discovered for this receptor type (orphan receptor). IRR, insulin receptor (IR), and insulin-like growth factor-I receptor (IGF-I-R) are all tyrosine kinases. The cellular function of the IRR is not known. The expression of IRR mRNA is restricted to a few, e.g. neuronal tissues, and has also been found in neuroblastomas. Since tyrosine kinase receptors, including the IGF-I-R, may be involved in tumor genesis, we examined the expression of IRR mRNA and IGF-I-mRNA in 18 tumor cell lines using RT-PCR and the solution hybridization/RNAse protection assay. In particular, the mRNA levels of IRR and IGF-I-R were compared by semi-quantitative RT-PCR in seven neuroblastomas and 11 soft tissue sarcomas (STS), five of which were of neuronal origin. In all of the seven neuroblastoma cell lines and in five of the 11 STS cell lines, the IRR mRNA was detected. In addition, the IRR mRNA was expressed in rhabdomyosarcoma, in leiomyosarcoma, in one of the Ewing sarcoma and in the neurofibrosarcoma cell line. The last two tumor cell types are of neuronal origin. The levels of expression of IGF-I-R and IRR mRNA of the neuroblastoma cell lines were closely related (r = 0.82, P < 0.002). Furthermore, IRR mRNA was found only in cell lines that also expressed IGF-I-R mRNA. In conclusion, cell lines from pediatric tumors of neuronal origin express IRR mRNA simultaneously with a another tyrosine kinase receptor (IGF-I-R) mRNA. The tight coupling of their mRNA expression suggests a functional association of both receptors in the tumor cells.
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PMID:Correlation of type I insulin-like growth factor receptor (IGF-I-R) and insulin receptor-related receptor (IRR) messenger RNA levels in tumor cell lines from pediatric tumors of neuronal origin. 1053 6