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 sarcomas, particularly those of soft-tissue origin, pose substantial diagnostic challenges for the clinician and pathologist. Several small round cell sarcomas, including Ewing's sarcoma, peripheral primitive neuroectodermal tumor, and alveolar rhabdomyosarcoma, can be difficult to distinguish from one another. These same sarcomas can be difficult to distinguish from other small round cell tumors, including non-Hodgkin's lymphoma and neuroblastoma. Spindle cell sarcomas, including malignant peripheral nerve sheath tumor, synovial sarcoma, and leiomyosarcoma, present similar diagnostic challenges. This review discusses 1) recent advances in immunohistochemistry, electron microscopy, and cytogenetics that enable a specific diagnosis in virtually all sarcoma cases; 2) cell biology and oncogenetic implications of novel morphologic and genetic findings in sarcomas; and 3) clinical implications of the recent characterization of several family cancer syndrome genes.
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PMID:Laboratory investigation, genetics, and experimental models in sarcomas. 193 25

Conventional chemotherapy results in high mortality rates in patients with solid tumors involving the bones or the bone marrow. High dose melphalan (MEL) with or without total body irradiation followed by bone marrow transplantation (BMT) has prolonged survival, but curative potential has remained disappointing. In order to improve survival 20 children with generalized or relapsed solid tumors (neuroblastoma, peripheral neuroectodermal tumor, Ewing's sarcoma, rhabdomyosarcoma) underwent autologous (n = 16) or allogeneic (n = 4) BMT. The myeloablative regimen consisted of 12 Gy fractionated total body irradiation (FTBI) and high dose MEL. In 12 of these patients this regimen was intensified by giving 60 mg/kg etoposide (1800 mg/m2 VP), and 1.5 g/m2 carboplatin (CBDCA) was added in seven of these 12 patients. The intensification of FTBI and MEL by adding VP and CBDCA was followed by acceptable toxicity. Acute liver toxicity in 15/20 patients (75%) and acute renal toxicity in 17/20 patients (85%) did not exceed WHO grade 1. The use of the conditioning regimen FTBI-MEL-VP-CBDCA during first chemotherapy response is a promising approach in treatment of children suffering from generalized solid tumors.
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PMID:Is treatment intensification by adding etoposide and carboplatin to fractionated total body irradiation and melphalan acceptable in children with solid tumors with respect to toxicity? 193 52

A case of malignant peripheral neuroectodermal tumor occurring during the course of a human immunodeficiency virus (HIV) infection is reported. The patient was a male homosexual who presented with a rapidly enlarging tumor of the posterior lower thoracic wall. By light microscopic examination the tumor was a small cell tumor showing occasional structures suggestive of Homer-Wright rosettes. The strong positivity for neuron-specific enolase and the neurosecretory granules indicated the neural differentiation of the tumor. Its precise nature was shown cytogenetically by the presence of the t(11;22) translocation, which distinguished it from the classical neuroblastoma.
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PMID:Ultrastructural, immunohistochemical, and cytogenetic study of a malignant peripheral neuroectodermal tumor in a patient seropositive for human immunodeficiency virus. 218 93

A peculiar human cell line (GI-ME-N) derived from the metastatic bone marrow of a 2-yr-old patient with stage IV neuroblastoma (NB) was extensively characterized. Cell-type-specific markers, tumorigenicity in nude mice, morphology, cytogenetics, and amplification/expression of the N-myc gene were evaluated. All metaphases presented the typical 1p deletion. Surface markers specific for NB cells, vimentin, and neurofilament proteins were all clearly detectable with immunofluorescence and/or western blot procedures. Moreover, it was found that GI-ME-N cells did not express N-myc oncogene or HLA class 1 antigens, and were not classified as peripheral neuroectodermal tumor cells. However, extremely short latency and survival times, comparable to peripheral neuroectodermal tumor cells, were observed in nude mice grafted with GI-ME-N. In addition, no correlations were observed in tumorigenicity of N-myc amplified (IMR32) versus unamplified (SK-N-SH GI-ME-N) human NB cell lines in nude mice. We conclude that N-myc amplification/expression do not correlate with the aggressiveness of human NB in athymic animals, which is not always explained by the peripheral neuroectodermal tumor cell nature of the malignant cells, either.
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PMID:A new human highly tumorigenic neuroblastoma cell line with undetectable expression of N-myc. 229 63

One case of a malignant peripheral neuroectodermal tumor of the thoracopulmonary region (Askin-Tumor) is reported. The tumor represents a distinct pathologic entity of neuroectodermal origin. The patient developed a metastasis in the upper jaw and in a lumbar vertebra. Morphologic and immunocytochemical findings of this extremely rare tumor were described and differential diagnosis from other soft-tissue round-cell sarcomas, such as Ewing's sarcoma and neuroblastoma are discussed. Problems associated with the clinical behavior and treatment are outlined. The prognosis in most cases of Askin-tumor is poor. The recommended therapy is radical resection, irradiation and combination chemotherapy containing anthracyclines and a high dose of alkylating agents.
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PMID:[Askin tumor. Rare neurogenic tumor with metastasis to mouth, jaw and face regions]. 256 Apr 15

Peripheral neuroepithelioma of soft tissue belongs to the group of peripheral neuroectodermal tumors (PNETs), but because of its clinical, biological, and morphological characteristics, it differs from other small, round-cell sarcomas that appear in children (neuroblastoma) or in the thoracopulmonary region (Askin's tumor) and bone (peripheral neuroectodermal sarcoma of bone). We report ten new cases of such PNET variety, based on their histologic, immunohistochemical, and electron microscopic findings. In all of these cases, the clinicopathologic correlations demonstrated high malignancy, with an ominous outcome in nine cases. The mean age of the patients was 32.6 years and there was a clear male predominance (eight men, two women). Histologically, the presence of Homer-Wright rosettes is mandatory for diagnosis, being complemented with positive immunohistochemistry for several neural immunomarkers using paraffin-embedded material. Neuron-specific enolase, E-36, HNK-1, and chromogranin neural markers proved to be positive in a high number of cases, but other markers (S-100 protein, synapto-physin, GFA protein, and neurofilaments [70 kilodalton]) were absent. Electron microscopy confirmed the presence of neural structures, both by scanning and transmission electron microscopy.
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PMID:Peripheral neuroectodermal sarcoma of soft tissue (peripheral neuroepithelioma): a pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas. 265 93

Eleven fine needle aspiration (FNA) biopsies were performed in five children with neuroblastoma, including one patient with peripheral neuroectodermal tumor of the thoracopulmonary region (Askin tumor). Cytologic features in conjunction with immunocytochemistry and electron microscopy on the aspirated material enabled us to make a primary diagnosis in four of the five patients and diagnose local recurrence and metastatic disease in three patients. There were no false-positive or false-negative cytologic diagnoses; therefore, diagnostic accuracy was 100%. FNA is an extremely useful technique for the primary diagnosis and management of neuroblastoma and excludes other small cell malignancies of children. The results of this study and literature review demonstrate that FNA cytology coupled with ancillary techniques of immunocytochemistry and electron microscopy is a rapid, safe, minimally invasive procedure which can aid in the diagnosis and management of patients with neuroblastoma without resorting to more aggressive diagnostic procedures in selective cases.
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PMID:Clinical utility of fine needle aspiration in the diagnosis and management of neuroblastoma. 335 32

Antibody-producing clones were obtained by hybridization of spleen cells from mice immunized with whole cultured human tumor cells and mouse myeloma cells, P3UX59AG8. The tumor cells were derived from a peripheral primitive neuroectodermal tumor. One of the antibodies produced by these clones reacts with the original cell line, SK-PN-DW, and other more differentiated neuroectodermal tumors such as neuroblastomas and melanomas. The cell line SK-PN-DW contains antigenic determinants recognized by monoclonal antibodies raised against melanoma, neuroblastoma, and human fetal brain. These data indicate that this primitive neuroectodermal tumor is derived from the neuroectoderm.
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PMID:Neuroectodermal tumor and monoclonal antibodies. 609 34

Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. Fifteen of the 38 previously reported cases have involved children from birth to 17 years of age. The authors observed the course of a 3-month-old girl who presented with an enlarging mass in the left arm and manifested hepatic metastases at the time of diagnosis. The urinary level of vanillylmandelic acid (VMA) was moderately elevated. The primary lesion was excised and metastatic foci showed response to a regimen of vincristine, cyclophosphamide, Adriamycin (doxorubicin), and cisplatin. However, tumor recurred in the brain and liver and the child died 14 months after diagnosis. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia and the liver contained numerous involuted lesions as well as active metastases. It is suggested that this is a unique neoplasm, derived from neural crest but distinct from neuroblastoma, which can be characterized by peripheral origin, a histologic pattern of confluent pseudorosettes, and aggressive clinical behavior.
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PMID:Peripheral neuroepithelioma in childhood. 649 81

The distinction of rhabdomyosarcoma (RMS) from other small blue round cell tumors of childhood, such as Ewing's sarcoma/peripheral primitive neuroectodermal tumor (pPNET) and neuroblastoma, continues to present a diagnostic challenge to pathologists. The recent recognition of the master role of myogenic regulatory proteins in skeletal muscle commitment and differentiation, and the availability of monoclonal antibodies to two of them (myogenin and MyoD1), has prompted us to test their diagnostic utility in routinely processed, formalin-fixed, and deparaffinized tissue. Preliminary studies had demonstrated that, with the use of heat-induced epitope retrieval techniques, expression of myogenin and MyoD1 could be documented specifically in nuclei of fetal skeletal muscle by the respective antibodies. We performed a retrospective immunohistochemical analysis on 72 cases of small blue round cell tumors, including 33 RMSs, 1 metastatic myogenous Wilms' tumor, 26 Ewing's sarcomas/pPNETs, and 12 neuroblastomas. Nuclear expression of myogenin and MyoD1 were both found in 30/33 non-overlapping cases of RMS, with no significant differences in the sensitivity with respect to histological subtypes, and in 1/1 case of myogenous Wilms' tumor. None of the neuroblastomas or Ewing's sarcomas/pPNETs demonstrated positive nuclear staining with either antibody. However, most of the neuroblastomas, and occasional Ewing's sarcomas/pPNETs, showed variable fibrillary, cytoplasmic immunoreactivity with antibody to MyoD1. We conclude that, with the use of microwave-based epitope retrieval, antibodies to myogenin and MyoD1 are both useful markers for the identification of RMS among other small blue round cell tumors of childhood, but antibodies to myogenin have technical advantages over those to MyoD1, as the latter may cross-react with an unknown cytoplasmic antigen in non-muscle cells and tumors.
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PMID:Expression of myogenic regulatory proteins (myogenin and MyoD1) in small blue round cell tumors of childhood. 749 4


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