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)

Peripheral neuroepitheliomas are rare malignant tumors of presumed neural crest origin, arising outside the central and sympathetic nervous system. We present herein a case of a young man with diffuse peripheral neuroepithelioma of the abdominal cavity. Although we twice attempted a debulking procedure on this patient, and treated him with alternating VIP-CAV combined chemotherapy, his disease showed a relentless course without responding to any treatment. The development of peripheral neuroepithelioma in the abdominal cavity is an extremely rare occurrence. These rare malignant tumors, of presumed neural crest origin, arise outside the central and sympathetic nervous systems (1). Other names for this tumor are: extracranial primitive neuroectodermal tumor (PNET), Askin tumor, peripheral or adult neuroblastoma (2,3,4). We present a case of a young man with diffuse development of this tumor in the abdominal cavity.
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PMID:Peripheral neuroepithelioma developing in the abdominal cavity. 892 Jul 88

Interferon alpha (IFN alpha) is used as an antineoplastic agent, both in hematopoietic malignancies and in solid tumors, because of its immunomodulatory action and direct antitumor activity. IFN alpha binds to specific cell-surface receptors that mediate its biologic activity. We studied the expression of IFN alpha receptors in pediatric solid tumors by use of the monoclonal antibody IFNaR3, which specifically recognizes the alpha subunit of the IFN Type I receptor. In three cell lines derived from those tumors, we determined the structure of the receptors by affinity cross-linking and immunoprecipitation techniques, and we determined their ability to mediate an antiproliferative effect. All of the tumor specimens studied by immunocytochemical analysis, including neuroblastomas, primitive neuroectodermal tumors, and rhabdomyosarcomas, stained positive for the IFN alpha receptor antibody, although in some cases immunoreactivity was weak. The three cell lines, derived from a neuroblastoma, a primitive neuroectodermal tumor, and a Ewing's sarcoma, respectively, showed the same pattern of IFN alpha receptor expression, both by affinity crosslinking and immunoprecipitation assays. Treatment with IFN alpha of those cell lines induces growth inhibition in vitro. These results suggest that IFN Type I receptor might be expressed in most solid tumors of childhood and that its structure is identical to the receptor expressed by the majority of hematologic malignancies.
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PMID:Expression of type I interferon receptor in solid tumors of childhood. 902 27

Fifteen children 4 years of age or under (8-46 months), weight 7.8 to 17 kg, underwent 44 peripheral blood stem cell (PBSC) collections. Diagnoses included PNET/medulloblastoma (five), neuroblastoma (five), and others (five). PBSCs were collected following G-CSF/GM-CSF or chemotherapy plus G-CSF/GM-CSF mobilization. All PBSC collections were well tolerated. The average yield per collection was 6.80 x 10(8) mononuclear cells/kg (1.1-30 x 10(8)/kg) or 57.60 x 10(6) CD34+/kg (1.37 to 480 x 10(6)/kg). Eight patients underwent stem cell transplantation following myeloablative chemotherapy. Six of the eight children who received PBSC following myeloablative therapy also received autologous bone marrow (0.7 to 3.6 x 10(8) MNC/kg). One heavily pretreated patient experienced delayed hematologic reconstitution, while the remaining seven patients had a median ANC recovery to > 0.5 x 10(3)/microliter by day +10 (9-11 days) and platelets > 50 x 10(3)/microliter by day +15 (12-17 days). Seven patients received PBSCs following repetitive submyeloablative chemotherapy (ICE: ifosfamide 1.8 g/m2/day, etoposide 100 mg/m2/day x 5, carboplatin 400 mg/m2/day x 2) or other similar combination chemotherapy. Median days to recover ANC > or = 1 x 10(3)/microliter and platelets > or = 100 x 10(3)/microliter in children receiving ICE + PBSCs were 10 and 14 days, respectively, compared with 16 and 22 days in children receiving ICE + G-CSF in historical controls. In conclusion, collection and use of PBSCs to support either myeloablative chemotherapy or multicycle submyeloablative chemotherapy is well tolerated and may enhance hematological recovery in young children and infants.
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PMID:Collection and use of peripheral blood stem cells in young children with refractory solid tumors. 902 45

Medulloblastoma is the most common primitive neuroectodermal tumor of the central nervous system, and shares some biochemical and immunological features with neuroblastoma. It could be suggested that N-myc expression in medulloblastoma is correlated with primitiveness or cell differentiation, as in neuroblastoma. N-myc expression in two human medulloblastoma cell lines (ONS-76 and -81) was investigated during drug-induced differentiation by immunocytochemistry and Western blotting. The growth rates of the two medulloblastoma cell lines showed a marked decrease and morphological differentiation occurred after treatment with 1 mM dibutyryl cyclic-adenosine 3',5'-monophosphate (dbt-cAMP). The decrease in N-myc expression with differentiation of cells was confirmed by immunocytochemistry and Western blotting. We believe that decreased N-myc expression is correlated with cell differentiation, and a decrease in the primitiveness of medulloblastoma cells.
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PMID:Decreased N-myc expression in human medulloblastoma cell lines during differentiation. 906 68

Although primitive neuroectodermal tumor (PNET) is a well-recognized entity, its renal localization as a primary site has not been appreciated. Only nine cases of renal PNET exist in the literature. The paucity of renal PNET could be explained by the lack of objective diagnostic techniques that would facilitate its distinction from other primitive round cell tumors of the kidney, such as the more widely recognized monophasic Wilms' tumor and clear-cell sarcoma of the kidney (CCSK), as well as renal carcinoid, or neuroblastoma invading the kidney from the adjacent adrenal gland. The recently identified specific fusion transcripts detectable by reverse transcription polymerase chain reaction (RT-PCR) have provided us with a valuable tool for the detection of renal PNET. This article reports three renal PNET that expressed EWS/FLI-1 fusion transcripts by RT-PCR, in addition to positive staining for MIC2 protein and neuron-specific enolase (NSE). One also exhibited dense core granules in cell processes by electron microscopy. Employment of such methodology will lead to a more accurate classification of renal tumors.
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PMID:EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. 922 42

Twenty-nine young men (mean age 29 years) had primitive neuroectodermal tumors (PNETs) arising in germ cell tumors (GCTs). Nine patients had PNETs confined to the testis, eight patients had PNETs in the testis and at metastatic sites, and 12 patients had PNETs identified only at extratesticular sites. Immunohistochemistry was of use in the further classification of these PNETs as neuroblastoma, medulloepithelioma, peripheral neuroepithelioma, or ependymoblastoma. The histologic pattern of PNETs in the testis (neuroblastoma or medulloepithelioma) did not predict which tumors metastasized. PNETs localized to the testis did not affect prognosis. Eight patients with no PNETs outside the testis were free of disease 1 month to 10 years after diagnosis. PNETs in extratesticular sites were an adverse prognostic factor. Nineteen patients with extratesticular PNETs had adequate clinical follow-up. Thirteen are dead of disease from 4 months to 5 1/2 years (mean 26 months) after diagnosis, four are alive with disease 6 months to 2 years after diagnosis, and two have no evidence of disease with short follow-up (6 and 17 months). Mean survival was longer (34 months) for patients whose extratesticular PNET was neuroblastoma than for those with other types of PNETs (13 months). Chemotherapy directed against GCTs was not effective in patients who developed metastatic PNETs of GCT origin. We conclude that extratesticular PNETs in patients with testicular GCTs are usually fatal, but patients with neuroblastomatous metastases may have a more prolonged course.
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PMID:Primitive neuroectodermal tumors arising in testicular germ cell neoplasms. 1007 29

The p21 protein inhibits the activity of cyclin-Cdk complexes and suppresses cell cycle progression. Wild type p53 can induce p21, but mutated p53 cannot. Previous studies have demonstrated that mutation of p53 is absent in neuroblastoma (NB). These reports prompted us to examine whether p53 induced p21 in NB. We examined the expression of p21 and p53 mRNA in eight NB, two Ewing's sarcoma (ES) and two primitive neuroectodermal tumor (PNET) cell lines by Northern blot analysis, and sequenced p53 cDNA of these cells. Although p53 mRNA was detected in all analyzed cell lines by Northern blot analysis, p21 mRNA was detected in six NB but not in two NB, two ES and two PNET cell lines. We detected the point mutation of p53 at codon 273 (CGT to TGT) in one NB and two ES cell lines. The non-transforming substitution at codon 72 (CCC to CGC) was detected in all analyzed cell lines. One PNET cell line had a large deletion of p53 cDNA. These results showed that p21 mRNA was usually expressed in NB but not in ES and PNET. This may suggest that the down stream of the p53 signal transduction pathway in NB is different from that of the closely related tumors of ES and PNET.
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PMID:p21 (WAF1/Cip1/Sdi1/Pic1) mRNA is expressed in neuroblastoma cell lines but not in Ewing's sarcoma and primitive neuroectodermal tumor cell lines. 936 58

Extraskeletal Ewing's sarcoma (EES) is a round-cell malignancy that manifests most commonly in the paravertebral and intercostal regions. It occurs predominantly in adolescents and young adults, between the ages of 10 and 30 yr, and follows an aggressive course with a high recurrence rate. Distant metastasis is also common. The tumor is often confused with other round, small-cell neoplasms, including primitive neuroectodermal tumor, neuroblastoma, embryonal rhabdomyosarcoma, and lymphoma. This report pertains to a fine-needle aspiration cytologic diagnosis of EES, supported by clinicopathologic and fine structural correlations in a 56-yr-old man who presented with a rapidly growing, massive, right groin mass. The smears showed a diffuse cellular population of malignant round cells composed of two types: one group of larger cell exhibiting a thin-rim, pale cytoplasm, less hyperchromatic nuclei, nucleoli, and diffusely dispersed chromatinic nuclear details; and the second group of smaller and darker cells with highly hyperchromatic and almost smudged nuclei. These are chief cells and dark cells, respectively. Special studies revealed significant intracytoplasmic glycogen and positive vimentin and HBA-71 immunostaining. Cytogenetic findings of chromosomal 11;22 translocation is also supportive of the diagnosis of EES.
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PMID:Fine-needle aspiration cytology of extraskeletal Ewing's sarcoma. 948 43

The resting energy expenditure (REE) and the respiratory quotient (RQ) were measured longitudinally using indirect calorimetry to examine the effects of total parenteral nutrition (TPN) on energy metabolism in children undergoing autologous peripheral blood stem cell transplantation (PBSCT). There were six children (two males and four females) and the age ranged from five to 13 years (median, eight yrs). The diagnosis included acute lymphocytic leukemia (ALL; 4), neuroblastoma (NBL; 1) and primitive neuroectodermal tumor (PNET; 1). TPN was started after the patients were stabilized following PBSCT (group A; n = 3) or before the initiation of high-dose cytoreductive chemotherapy (HCC) (group B; n = 3). Duration of HCC before PBSCT was identical between the two groups (six to eight days). Average total calorie and protein intake during HCC was significantly higher for group B than for group A. The %REE, the percentage of REE to the predicted basal energy expenditure (BEE), in group A showed 133 +/- 19%, 129 +/- 14% and 146 +/- 11% during three periods of HCC (days -8 to -1 of PBSCT), bone marrow suppression (days 0 to 11 of PBSCT) and bone marrow recovery (days 12 to 22 of PBSCT), respectively. In contrast, those in group B were 10% to 20% lower than those in group A at all periods. Carbohydrate oxidation rates during HCC in group A were significantly lower than those in group B, and those were not different between both groups during post-PBSCT periods. Fat oxidation rates in both groups were similar at all stages of periods. In contrast, protein degradation rates in group A were significantly higher than those in group B at all stages of the period. From these results, we concluded that commencement of TPN administration prior to HCC in the patients undergoing PBSCT provides beneficial effects to maintain better energy metabolic and nutritional status.
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PMID:Total parenteral nutrition on energy metabolism in children undergoing autologous peripheral blood stem cell transplantation. 959 9

The purpose of this study was to provide an overview of the spectrum of pediatric chest masses, to present the results of cross-sectional imaging with CT and/or MRI, and to define diagnostic criteria to limit differential diagnosis. Seventy-eight children with thoracic mass lesions were retrospectively evaluated using CT (72 patients) and/or MR imaging (12 patients). All masses were evaluated for tissue characteristics (attenuation values or signal intensity, enhancement, and calcification) and were differentiated according to age, gender, location, and etiology. Twenty-eight of 38 (74 %) mediastinal masses were malignant (neuroblastoma, malignant lymphoma). Thirty of 38 (79 %) pulmonary masses were metastatic in origin, all with an already known primary tumor (osteosarcoma, Wilms tumor). With one exception, all remaining pulmonary lesions were benign. Seventeen of 21 (81 %) chest wall lesions were malignant (Ewing sarcoma, primitive neuroectodermal tumor). The majority of mediastinal and chest wall tumors in children is malignant. Lung lesions are usually benign, unless a known extrapulmonary tumor suggests pulmonary metastases. Cross-sectional imaging with CT and/or MRI allows narrowing of the differential diagnosis of pediatric chest masses substantially by defining the origin and tissue characteristics. Magnetic resonance imaging is preferred for posterior mediastinal lesions, whereas CT should be used for pulmonary lesions. For the residual locations both modalities are complementary.
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PMID:Cross-sectional imaging with CT and/or MRI of pediatric chest tumors. 968 16


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