Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
 ...
PMID:Cytogenetic and pathologic aspects of Ewing's sarcoma and neuroectodermal tumors. 133 Aug 77

The group of rounded small-cell tumors include different neoplasias involving different therapies; we can name neuroblastoma, Ewing' sarcoma, embrionary rhabdomyosarcoma, lymphoma and other pathology such as Askin's tumor or small cell tumor of the thorax area. Based on microscopic and immunohistochemistry findings, it is suggested that it originates from the neural crest or pluri-potential cells from the neuroectodermy. This has a very aggressive behaviour and is usually resistant to oncologic therapies.
 ...
PMID:[Undifferentiated small cell tumor of the thoracopulmonary region]. 195 81

The histogenesis of Ewing's sarcoma (EW) and extraskeletal Ewing's sarcoma (EEW) is still disputable. Their relationship to the so-called Askin's tumor, neuroectodermal tumor of bone, and peripheral neuroblastoma remains to be established. In an attempt to clarify these points, immunocytochemical and ultrastructural studies were done on tissues from 14 cases of EW, 4 cases of EEW, and 9 cases of primitive neuroectodermal tumor (PNET) and compared with neuroblastoma and olfactory neuroblastoma. Six tumors categorized initially as EW and EEW on biopsy, turned out to be PNET by extensive histologic and/or ultrastructural observations. Abundant glycogen was recognized not only in 16 of 18 cases of EW and EEW, but also in seven of nine cases of PNET. Fine fibrillar cell processes were seen between tumor cells, at least in limited areas even in cases of EW and EEW. Immunocytochemically, neuron-specific enolase (NSE), neuroblastoma cell surface antigen (NBCA), neuron cell surface antigen (NCSA), and neurofilament (NF) were demonstrated not only in neuroblastoma, but also frequently in cases of EW, EEW, and PNET. The results seem to suggest that EW and EEW represent the most immature forms of neuroectodermal tumor. Electron microscopic study showed predominantly primitive cells with occasional areas of cell processes, neurosecretory granules, and microtubules, suggesting a neuroectodermal origin.
 ...
PMID:Immunocytochemical and ultrastructural studies of the histogenesis of Ewing's sarcoma and putatively related tumors. 254 95

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.
 ...
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

Small, round, blue-cell tumors (SRCT), including rhabdomyosarcoma, Ewing's sarcoma of bone and soft tissue, mesenchymal chondrosarcoma, small cell osteosarcoma, hemangiopericytoma, neuroblastoma, peripheral neurectodermal tumor (peripheral neuroepithelioma of bone and soft tissue), and the malignant small cell tumor of the thoracopulmonary region described by Askin (Askin's tumor), are often difficult to distinguish by light microscopy. We have evaluated the cytogenetics of these tumors by studying 24 tumor explants in short-term culture and 22 tumor cell lines. In Ewing's sarcoma (a tumor of unknown histogenesis), and in peripheral neuroepithelioma and Askin's tumor (tumors with evidence of neural origin), we have observed an indistinguishable t(11;22) translocation.
 ...
PMID:Cytogenetic characterization of selected small round cell tumors of childhood. 300 99

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.
 ...
PMID:Clinical utility of fine needle aspiration in the diagnosis and management of neuroblastoma. 335 32

Neurogenic tumors of the thorax are observed in all age groups, although they are rare in the elderly. They are more likely to be malignant in the child than in the adult. Tumors of the autonomic system are common in children, whereas the nerve sheath tumors are more likely to be found in adults. The malignant lesions are almost always symptomatic and the benign lesions asymptomatic, except in the child, in whom a benign lesion may result in symptoms because of its size relative to the volume of the child's thorax. Intraspinal canal extension, although relatively infrequent, should be sought for in all paravertebral tumors, for a significant percentage of these may be initially asymptomatic. Excision of such an hourglass tumor without foreknowledge of such extension may lead to serious spinal cord complications. Surgical excision of most of these tumors is sufficient except for the malignant lesions, especially in infancy and childhood, for which the addition of postoperative irradiation and chemotherapy may be beneficial. The prognosis after the removal of benign neurogenic tumors of the thorax is excellent. In the infant or child with Stage III or IV neuroblastoma, ganglioneuroblastoma, or an Askin tumor, the prognosis is poor. In the adult with a malignant neurogenic sarcoma or a malignant paraganglioma, the prognosis likewise is grave.
 ...
PMID:Neurogenic tumors of the thorax. 337 59

Malignant small cell tumor of the thoracopulmonary region (MSCT) was first described in 1979 and has been referred to as the Askin tumor. This malignant neoplasm is a member of the peripheral primitive neuroectodermal tumor (PPNET) family and typically involves the periosteum, soft tissue, and extrapulmonary tissue of the thoracic wall. MSCT may also involve the lung parenchyma by local extension or may arise de novo in peripheral lung tissue. Local recurrence, abdominal involvement by tumor extravasation across the diaphragm, and skeletal metastatic disease are relatively common. However, metastasis to the head and neck region and in particular to the oral cavity is extremely rare. We present a recurrent intrapulmonary MSCT with metastasis to the oral cavity in an adolescent Hispanic boy, and review the literature regarding this member of the PPNET family. Differentiation from neuroblastoma may be made based on immunoreactivity for beta 2 microglobulin and HBA71 and lack of immunoreactivity for chromogranin in PPNET and MSCT. Ultrastructural features commonly seen in MSCT and PPNET are round to ovoid tumor cells with occasional cytoplasmic processes with relatively few pleomorphic dense core granules. These tumors lack the gangliocytic and Schwann cell differentiation that is characteristic of neuroblastoma. MSCT and PPNET have a common reciprocal cytogenetic translocation [t(11;22)q(24;q12)], which is shared with Ewing's sarcoma. Prognosis in MSCT is quite dismal, with a 2-year survival of 38% and a 6-year survival of only 14%.
 ...
PMID:Recurrent intrapulmonary malignant small cell tumor of the thoracopulmonary region with metastasis to the oral cavity: review of literature and case report. 757 Oct 88

Malignancies of childhood include a well-defined spectrum of hematolymphoid, organ specific (adrenal, kidney, liver), soft tissue, bone, and nervous system (central and peripheral) neoplasms with variable biology. Small round cell neoplasms, a subset of childhood malignancies, are histologically similar but differ markedly in their histogenesis, therapy, and prognosis. Traditionally, clinical information and light microscopy, with the aid of histochemistry and ultrastructural evaluation, establish a diagnosis or at least narrow the differential diagnosis. Additionally, immunohistology, cytogenetics, and molecular studies have become important in diagnosis and in defining phenotype/genotype, patient treatment modalities, and prognosis in specific cases. The 11;22 chromosomal translocation typifies Ewing's sarcoma, primitive neuroectodermal tumor, and Askin's tumor, as does the resultant chimeric transcript, while expression and amplification of N-myc oncogene are predictive of the prognosis in neuroblastoma. Furthermore, studies of genes and gene products are elucidating mechanisms of oncogenesis and tumor progression.
 ...
PMID:Immunohistology, cytogenetics, and molecular studies of small round cell tumors of childhood. A review. 776 71

A comparative study concerning the histogenesis of Ewing's sarcoma and its relationship with peripheral primitive neuroectodermal tumor (PNET) was undertaken using histological, immunohistochemical and ultrastructural techniques. The cases studied included 22 Ewing's sarcomas, 17 (ganglio) neuroblastomas, 3 peripheral PNETs, 1 peripheral neuroblastoma, 1 Askin's tumor and a case in which it was difficult to distinguish between Ewing's sarcoma and neuroblastoma. Almost all the cases of Ewing's sarcoma showed neural differentiations. Immunohistochemical study using antibodies for neuron-specific enolase, protein gene product 9.5, Leu 7 and neurofilament (200 kD) was most useful for examining the neural differentiation of these tumors. These results suggest that Ewing's sarcoma is in a broad sense one of such peripheral PNETs as (ganglio) neuroblastoma, peripheral neuroblastoma and Askin's tumor.
 ...
PMID:[Clinicopathological study of Ewing's sarcoma and primitive neuroectodermal tumor]. 830 11


1 2 3 Next >>