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)

Plasma carcinoembryonic antigen (CEA) was assayed with a radioimmune procedure in 27 healthy control children. The upper limit of plasma CEA (mean +2 SD) was derived from healthy controls and was 3.35 ng/ml. This value was compared with those obtained from 15 children with active neuroblastoma, 7 with active embryonal rhabdomyosarcoma, 16 with treated neuroblastoma and without evidence of disease, 14 disease-free patients with embryonal rhabdomyosarcoma, and 17 patients still on therapy. The neuroblastoma and embryonal rhabdomyosarcoma patients with active disease had higher CEA values than did the successfully treated neuroblastoma and embryonal rhabdomyosarcoma patients. CEA plasma values greater than 3.35 ng/ml were found in 35% and 24% of patiens with neuroblastoma and embryonal rhabdomyosarcoma, respectively.
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PMID:Carcinoembryonic antigen in children with neuroblastoma. 97 77

A full-term black boy had a 2- to 3-cm, round, bluish mass on his right lower eye-lid at birth, later diagnosed as rhabdomyosarcoma. It was cystic in nature and extended into the nasal cavity. The tumor was initially classified as neuroblastoma. The child died eitht months later and necropsy report confirmed an original ophthalmologic pathology diagnosis of embryonal rhabdomyosarcoma.
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PMID:Rhabdomyosarcoma of the orbit in the newborn. 120 95

Diagnostic classification of poorly differentiated, round cell, primitive neuroectodermal neoplasms, including Ewing's sarcoma, peripheral neuroepithelioma, Askin's tumor, and esthesioneuroblastoma, is challenging to the surgical pathologist using conventional histopathologic approaches because of very similar and overlapping morphologic and cytologic features. Furthermore, distinguishing these neoplasms from neuroblastoma, embryonal rhabdomyosarcoma, small cell osteogenic sarcoma, and non-Hodgkin's lymphoma can be difficult. This paper describes and reviews the cytogenetic and molecular genetic changes in these tumors and demonstrates how the ability to detect these changes has enabled a greater understanding of the histogenesis, classification, diagnosis, and prognosis of these neoplasms.
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PMID:Cytogenetic and pathologic aspects of Ewing's sarcoma and neuroectodermal tumors. 133 Aug 77

Peripheral pancytopenia is a syndrome which allows for an early diagnosis, and although is may cover a large number of pathological entities, it can be clearly defined into three groups of illnesses which evolve with this syndromal manifestations. The first group includes non-neoplastic illnesses which include aplastic anemia, hemophagocytic syndrome associated to infection, immunological diseases and the deficiency of folates or vitamin B12. The second group includes neoplastic diseases as acute leukemia, non-Hodgkin lymphoma, and Hodgkin's lymphoma with myelofibrosis, malignant histiocytosis and non-hematological neoplasms, like the neuroblastoma and the embryonal rhabdomyosarcoma. The third group is formed by illnesses which have some similarity with neoplasms.
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PMID:[Peripheral pancytopenia]. 228 61

A parent rhabdomyosarcoma cell line designated SCMC-RM2 was established from bone-marrow tumor cells taken from an 11-year-old girl with an embryonal rhabdomyosarcoma. Subsequently a cloned SCMC-RM2-1 cell line was isolated from a parent line. These cell lines grew as adherent monolayers in liquid culture with a doubling time of 50 and 52 hr, respectively. In addition, colonies were established in soft agar, which grew in a dose-dependent fashion with a cloning efficiency of 0.7 and 0.8%, respectively. Chromosomal analysis showed these cell lines had neither double minutes nor homogeneously staining regions. Chromosome number ranged from 61 to 93, translocation; t(9;13)(p22;q14) was identified, and no alteration of chromosome 2 was observed. Surface membrane antigen profile of parent and cloned lines by using a panel of 24 monoclonal antibodies (MAbs) excluded the possibility of these being neuroblastoma cell lines. In addition, MAbs to the cytoplasmic protein desmin, myoglobin, muscle actin (alpha and gamma) and alpha-sarcomeric actin reacted with these cell lines, SCMC-RM2 and SCMC-RM2-1 being thus identified as rhabdomyosarcoma. Southern blot analyses revealed 8- and 7-fold amplification of the N-myc gene in SCMC-RM2 and SCMC-RM2-1 as compared with the promyelocytic cell line HL60. Over-expression of the N-myc mRNA was noted over control cell lines.
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PMID:Characterization of an embryonal rhabdomyosarcoma cell line showing amplification and over-expression of the N-myc oncogene. 232 48

The authors have examined extracellular matrix (ECM) biosynthesis by small round cell tumors of childhood. Basal lamina (laminin and Type IV collagen) and stroma (collagens I, III, and V and fibronectin) constituents were studied. It was found that these tumors synthesize ECM in characteristic patterns. Five Ewing's sarcomas variably synthesized small amounts of all ECM constituents except Type V collagen. All eight neural tumors (neuroblastoma and primitive neural tumors) synthesized fibronectin (unlike some Ewing's sarcomas), as well as laminin and Type IV collagen (2 cases lacked Type IV collagen synthesis). No stromal (I/III) collagen synthesis was observed by neural tumors. All soft tissue sarcomas except an embryonal rhabdomyosarcoma synthesized stromal collagens and often laminin or fibronectin as well. Lymphomas synthesized no ECM of any kind. The synthesis of stromal collagens by sarcomas but not neural tumors serves to distinguish these two tumor types, especially Ewing's sarcoma from neuroblastoma. The presence of any ECM synthesis excludes lymphoma from diagnostic consideration.
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PMID:Extracellular matrix synthesis by undifferentiated childhood tumor cell lines. 282 16

A case of extrarenal malignant rhabdoid sarcoma arising in the pelvic soft tissues of a 12-year-old girl is described. By routine light microscopy the tumour resembled, in some areas, an embryonal rhabdomyosarcoma and, in other areas, a neuroblastoma. Electron microscopy revealed characteristic cytoplasmic aggregates of intermediate filaments, often with central clusters of organelle membranes surrounded by these filaments. Immunohistochemical stains showed strong cytoplasmic reactivity for vimentin. Staining for cytokeratin, myoglobin, desmin, neurofilaments, neurone specific enolase, S-100 protein and leucocyte common antigen was negative. A histogenetic origin from primitive mesenchymal cells is favoured. We strongly support the use of electron microscopy for the definitive diagnosis of small round cell undifferentiated sarcomas of childhood.
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PMID:Malignant rhabdoid tumour of soft tissue. An ultrastructural and immunohistological study of a pelvic tumour. 357 Jan 77

Small round cell neoplasms, which include neuroblastoma, Ewing's sarcoma, embryonal rhabdomyosarcoma, oat cell carcinoma, and lymphoma, are often confused with one another histologically. The advent of successful but distinct therapeutic approaches for different neoplasms has increased the need for precise diagnosis. The use of techniques ancillary to routine histologic or ultrastructural analysis allows better definition of the specific tumor type. Four cases of small cell tumor are described in which the initial working diagnosis was incorrect. The application of two diagnostic procedures for neuroblastoma was of great value to clarifying the proper diagnosis. These tests were a rapid fluorescence assay for intracellular catecholamines and a tissue culture assay for neurite outgrowth. Both of these methods are highly sensitive for neuroblastoma and distinguish neuroblastoma from other small round cell neoplasms. Their use confirmed this unsuspected diagnosis in three cases and excluded neuroblastoma in the fourth case.
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PMID:The diagnostic dilemma of the "small round cell neoplasm": catecholamine fluorescence and tissue culture morphology as markers for neuroblastoma. 627 91

In its histologic features, embryonal rhabdomyosarcoma (RMS), the prototype of malignant soft tissue tumors in childhood, summarizes the problems associated with the diagnosis of this entire group of neoplasms. Many of the tumors that do not fulfill the criteria for RMS have been designated "sarcomas of uncertain histogenesis." The introduction of the concept of a soft tissue equivalent of Ewing's sarcoma may have eased the semantic anxiety without improving our conceptual understanding. It is thought that the embryonal RMS, Ewing's sarcoma, and other are derived from a primitive mesenchymal cell. Another separate category of "small blue cell tumors" are those which presumably originate from the primitive neuroepithelium. Some of the diagnostic terms applied to this category are "neuroepithelioma," "medulloepithelioma," and "peripheral neuroblastoma." Because most of these tumors are hormonally inactive and electron microscopy is not performed, the diagnosis is infrequently considered or proved. The recently described small cell tumor of thoracopulmonary origin is likely a malignant neuroepithelial neoplasm. Hematopoietic tumors, such as non-Hodgkin's malignant lymphomas, granulocytic sarcoma, and malignant histiocytosis, may appear in the soft tissues as the initial manifestation of these system diseases. A final group of malignant soft tissue tumors are the fibrohistiocytic ones with a biphasic pattern of small round cells and spindle cells. It now has become increasingly difficult for the pathologist to satisfy his clinical colleagues with the diagnosis of "undifferentiated malignant tumor" in a child.
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PMID:Soft tissue sarcomas of childhood: the differential diagnostic dilemma of the small blue cell. 627 17

Morphological transformation of NIH/3T3 cells by transfection with DNA has been used to identify transforming sequences in human tumours. Transforming activity has been reported for DNAs isolated from bladder, mammary, colon and lung carcinomas, neuroblastoma, lymphoid and myeloid tumours. Each of these tissues seems to contain different transforming sequences except for the colon and lung tumours where the same sequence seems to be involved. We now report that in two different human sarcoma cell lines, a fibrosarcoma and an embryonal rhabdomyosarcoma, the DNAs have transforming activity. The transforming gene is the same in both sarcomas but differs from the activated sequences detected in other tumours. We have also found that the transforming gene has no detectable homology to eight retrovirus oncogenes tested.
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PMID:A transforming gene present in human sarcoma cell lines. 628 87


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