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)

During the last 11 years, 144 nonlymphoreticular neoplasms were diagnosed in exfoliative cytology specimens obtained from patients younger than 17 years of age. Neuroblastoma was the single most common neoplasm (30 cases). Other categories of malignant neoplasms were primary bone tumors (30 cases), soft-tissue sarcomas (25 cases), brain tumors (25 cases) and epithelial neoplasms (7 cases). Of the 780 cytologic specimens, 335 were positive for malignant cells. Serous effusions provided most of the positive specimens from patients with neuroblastoma, germ-cell tumors and bone sarcomas. Exfoliated cells of metastatic embryonal rhabdomyosarcoma and primary brain tumors were detected most often in cerebrospinal fluid specimens. A most unusual presentation of an immature teratoma of the ovary is described in some detail. Despite the rarity of pediatric neoplasms, certain specific or suggestive cytologic features were recognized, including rosette formation of neuroblasts, nuclear notching of myoblasts, pleomorphism of osteoblasts and fibrillar processes of glial elements.
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PMID:Exfoliative cytology of nonlymphoreticular neoplasms in children. 632 May 62

This paper presents the case report of a 2 year-old boy in whom a grade II fetal alcohol syndrome was diagnosed at 17 months. At 21 months, an embryonal rhabdomyosarcoma of the urinary bladder trigone was found, and the tumour excised. Polychemotherapy was initiated, but bone-marrow depression and death in septicaemic shock followed. The main autopsy findings included the demonstration of tumour remnants in the urinary bladder and prostate, and a severe acute purulent pyelonephritis. This is the fifth case of fetal alcohol syndrome in conjunction with a malignant tumour to be reported in the international literature. These cases do not show any uniformity as to tumour type. No conclusion can as yet be drawn as to whether there is a pathogenetic connection between the fetal alcohol syndrome and the occurrence of malignant tumours (as is the case with the fetal hydantoin syndrome and neuroblastoma), or whether these findings are purely coincidental. Clinicians and pathologists should, however, be advised to look more closely for even minimally pronounced forms of the fetal alcohol syndrome in children with embryonic tumours.
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PMID:[Fetal alcohol syndrome and malignant tumors]. 689 Jul 44

H19 and insulin-like growth factor II (IGF2) are among a few genes which have been confirmed to be imprinted in normal human embryonal tissues. This results in monoallelic expression of maternal H19 and paternal IGF2. Loss of imprinting of these genes producing biallelic expression has been observed in Wilm's tumor and embryonal rhabdomyosarcoma, suggesting that an epigenetic change of DNA, in addition to a genetic change in oncogene(s) and/or tumor suppressor gene(s), may be involved in the development of these childhood cancers. Neuroblastoma, which is an embryonal tumor originating from neural crest-derived cells, occasionally occurs in individuals with the Beckwith-Wiedemann syndrome; Wilm's tumor and embryonal rhabdomyosarcoma occur even more frequently in the Beckwith-Wiedemann syndrome; and paternal uniparental disomy of H19 and IGF2 loci (chromosome 11p15) is present in the Beckwith-Wiedemann syndrome. Furthermore, neuroblastoma cell lines express IGF2, and autocrine/paracrine effects of IGF2 have been demonstrated in these cells. Thus, we examined for imprinting of both H19 and IGF2 in primary untreated neuroblastomas using the RsaI and ApaI polymorphisms within these genes, respectively. Seven of 15 tumors were informative for H19 and for IGF2, and all of these cases showed monoallelic expression of both of these genes. These results indicate that loss of imprinting of H19 and IGF2 does not occur in neuroblastomas.
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PMID:Maintenance of normal imprinting of H19 and IGF2 genes in neuroblastoma. 761 76

Sixty-seven childhood tumors were studied immunohistochemically for the extracellular matrix element type IV collagen, laminin, and fibronectin. Tumors included Ewing's sarcoma, primitive neuroectodermal tumor, small cell osteosarcoma, neuroblastoma or ganglioneuroblastoma, rhabdomyosarcoma, and lymphoma. It was found that small cell osteosarcoma was often positive for fibronectin but not type IV collagen or laminin, a new observation. In the lymphomas, matrix proteins were rarely found. Ewing's sarcoma was variably positive for type IV collagen and laminin, but fibronectin was absent. Extracellular laminin and fibronectin were found in one of two cases of primitive neuroectodermal tumor. In neuroblastoma and ganglioneuroblastoma, the matrix components were rarely found. These results, discrepant with findings in cultured cells, may reflect the altered capacity of tumors to produce these proteins in vitro, which suggests that caution should be exercised in drawing conclusions regarding the nature or histogenesis of tumors from data obtained with cultured tumor cells. Embryonal rhabdomyosarcoma frequently contained all matrix elements in the extracellular space and in a dotlike pattern in the cytoplasm; alveolar rhabdomyosarcoma rarely contained these proteins and never exhibited the dotlike pattern. The frequent finding of matrix proteins in embryonal rhabdomyosarcoma but only rarely in alveolar rhabdomyosarcoma and the unique immunostaining pattern in embryonal rhabdomyosarcoma may prove to be a useful adjunct in the diagnosis of childhood tumors.
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PMID:Extracellular matrix of small round cell tumors of childhood: an immunohistochemical study of 67 cases. 815 9

Ewing's sarcoma is a very rare tumor which has, however, attracted much oncological interest since the dramatic improvement of its prognosis under chemotherapy. Its histogenesis has been discussed controversially for a long time, including a possible origin in immature reticulum, myogenous, endothelial and undifferentiated mesenchymal cells. Repeated reports have also suggested a possible neuroectodermal genesis. Convincing arguments, however, have only been brought forward during recent years, since it was found out that Ewing's sarcoma and malignant peripheral neuroectodermal tumor share a common chromosome translocation 11;22. In the meantime this hypothesis has been strengthened by numerous cell biological analyses. Histological differential diagnosis of Ewing's sarcoma has been improved by immunohistological methods. In most cases, they can be distinguished from lymphoma (leucocyte common antigen, B and T markers) and embryonal rhabdomyosarcoma (muscle specific actin, desmin). Apart from this, we now have an antibody specific for Ewing's sarcoma and malignant peripheral neuroectodermal tumors but not with neuroblastoma. Recent investigations regarding the prognosis under chemotherapy have shown that tumors with neural differentiation have a comparatively poor prognostic outcome. This is why in addition to the demonstration of Homer Wright pseudo-rosettes different neural markers such as neuron specific enolase, synaptophysin and chromogranin are to be analysed. Small cell osteosarcoma is a very rare sub-entity of osteosarcomas. Recent studies have shown that the tumor resembles Ewing's sarcoma with partial mesenchymal differentiation including osteoid formation.
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PMID:Round cell tumours of bone. 818 32

12E7 is a monoclonal antibody to the MIC2 gene product and can be applied to formalin-fixed, paraffin-embedded tissue. The diagnostic utility of 12E7 as a marker of Ewing's sarcoma and peripheral neuroectodermal tumour was assessed. Immunocytochemical studies were performed on 120 small round-cell tumours from children and adolescents. Immunoreactivity for 12E7 was seen in 13 of 15 Ewing's sarcomas. 14 of 15 peripheral neuroectodermal tumours, four of 14 embryonal rhabdomyosarcoma, seven of 11 T-lymphoblastic lymphomas and one T-cell acute lymphoblastic leukaemia. Immunoreactivity was located on the cell-membrane of Ewing's sarcomas, peripheral neuroectodermal tumours and lymphoid tumours while rhabdomyosarcomas showed weak, cytoplasmic staining in differentiated rhabdomyoblasts. Studies on alveolar rhabdomyosarcomas (n = 10), acute myeloid leukaemias (3), B-lymphoblastic lymphomas (8), blastema-rich nephroblastomas (9), neuroblastomas (20) and retinoblastomas (10) as well as single examples of B-cell acute lymphoblastic leukaemia, Ki-1 anaplastic lymphoma of indeterminate phenotype and intra-abdominal desmoplastic tumour with divergent differentiation were negative. 12E7 is a sensitive marker for the Ewing's sarcoma/peripheral neuroectodermal group of tumours and is useful in distinguishing them from neuroblastoma and blastema-rich nephroblastoma. However, immunopositivity for 12E7 should be interpreted in conjunction with the results of neural and lymphoid markers.
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PMID:Immunocytochemical study of 12E7 in small round-cell tumours of childhood: an assessment of its sensitivity and specificity. 831 40

Malignant ectomesenchymoma is a rare soft tissue tumor of the childhood believed to arise from a remnant of pluripotential migratory neural crest cell (ectomesenchym) and composed of both a mesenchymal element (most often embryonal rhabdomyosarcoma) and a neuroectodermal element (ganglioneuroma, schwanomma neuroblastoma or melanocytic cells). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. Herein we report a new case of an orbital ectomesenchymoma studied by means of histochemistry and immunohistochemistry in order to increase the morphologic and histogenetic knowledge of this peculiar tumor and its significance concerning the differential diagnosis.
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PMID:Primary malignant ectomesenchymoma of the orbit. 906 87

Solid malignant tumours (n = 263) excluding brain and spinal cord tumours in children up to 14 years of age were studied. Retinoblastoma (27%) constituted the largest group followed by Wilms' tumour (14.1%) and lymphoma (13.7%). Most patients (55%) were of less than 5 years age and maximum incidence of embryonal tumours was found in this age group; other tumours were more frequent in higher age. A male preponderance was noted (male to female ratio as 1.6:1). Amongst lymphoma, 61% were non-Hodgkin's lymphoma and rest were Hodgkin's disease; 2 cases of Burkitt's lymphoma were found. Other notable tumours encountered in the study were embryonal rhabdomyosarcoma (n = 14), hepatoblastoma (n = 9), neuroblastoma (n = 7), Ewing's sarcoma (n = 21), osteogenic sarcoma (n = 19) and germ cell tumours (n = 14).
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PMID:Pattern of solid malignant tumours in children--a ten-year study. 935 72

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

Approximately 30% of malignant neoplasms in children are dysontogenetic tumors whose pathologic features resemble or recapitulate those of the developing organ or tissue of origin. Archetypes include classic neuroblastoma, Wilms' tumor, and embryonal rhabdomyosarcoma. This review traces the history of the principal types of dysontogenetic neoplasms and the primitive round cell tumors, Ewing's sarcoma, and peripheral primitive neuroectodermal tumor. Retinoblastoma, neuroblastoma, and Wilms' tumor were first described in the 19th century but with several different appellations than those we use today. Although some progress was made in the surgical management of Wilms' tumor during the 1940s and 1950s, most of these unique solid neoplasms of childhood were seen as essentially untreatable and inevitably fatal; surgery and perhaps irradiation were the principal therapeutic offerings. The folic acid analogue, aminopterin, was reported in 1948 as inducing the first complete but temporary remission in acute childhood leukemia. The chemotherapeutic era began shortly thereafter with effective chemotherapy in the management of Wilms' tumor with the introduction of dactinomycin. Pathologists were no longer restricted to being purveyors of the death sentence; they were now responsible for differentiating one type of primitive and embryonic neoplasm from another by using a variety of ancillary techniques, including tissue culture, electron microscopy, immunohistochemistry, and cytogenetics. Favorable or unfavorable morphologic types and subtypes of tumors were defined and, together with the pathologic staging, became incorporated into the therapeutic plan and prognostic assessment. During the past 40 years, these tumors progressed from being virtually treatment resistant to having an overall 5-year survival of 70% or greater. Through the cooperative efforts of pediatric hematologists/oncologists, pediatric surgeons, radiation therapists, and pathologists, the primitive and embryonic neoplasms of childhood are now viewed as some of the most treatable and curable of cancers.
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PMID:The evolution of the diagnosis and understanding of primitive and embryonic neoplasms in children: living through an epoch. 968 89


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