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)

We here present a review of ten pediatric patients with rib tumors that were treated in our hospital since 1970: a juvenile bone cyst, an aneurysmal bone cyst, an eosinophilic granuloma, a Ewing sarcoma, a metastasis of a neuroblastoma, an osteochondroma, a hemangiopericytoma, and a callous tumor at the site of a previous single rib fracture. The relative frequency of rib tumors is similar to that of bone tumors in other locations. A biopsy of the affected bone should be the first step of the operative intervention. As demonstrated in a case of eosinophilic granuloma, the indication for subperiosteal rib resection can be defined generously. A completed regrowth of the rib defect can be expected. Even the loss of a complete rib is generally well tolerated, as shown in an infant with an aneurysmal bone cyst, a rare disease in this age group. Malignant tumors of the chest wall, however, require special experience in thoracic surgery and necessitate close interdisciplinary cooperation. Complete primary resection of a malignant rib tumor and its plastic reconstruction is demonstrated in a case of a Ewing sarcoma in childhood. In children and adolescents a vicryl net should generally be used to reconstruct extended chest wall defects reliably and functionally stable.
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PMID:[Tumors of the ribs in children]. 208 62

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.
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PMID:[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]. 267 76

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.
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PMID:Cytogenetic characterization of selected small round cell tumors of childhood. 300 99

The medical records of 973 previously untreated patients diagnosed between January 1960 and December 31, 1978 with childhood cancer were reviewed. Siblings in 13 families were diagnosed with cancer 9/12 to 15 years after the diagnosis of cancer in the index sibling. Previously unreported association of acute lymphoblastic leukemia with Hodgkin's disease, neuroblastoma with malignant hemangiopericytoma, non-Hodgkin's lymphoma with malignant melanoma, Wilms' tumor with non-Hodgkin's lymphoma, Hodgkin's disease with malignant teratoma of the testis and craniopharyngioma with acute myeloblastic leukemia were identified. Two families appeared to transmit a predisposition to childhood tumors. The data from these families extend previous observations regarding multiple cases of cancer in sibships.
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PMID:Childhood cancer in siblings. 315 35

A series of 129 soft tissue sarcomas was examined ultrastructurally to determine in which neoplasms and to what extent myofibroblasts could be demonstrated. Twenty cases of fibromatosis and fasciitis served as controls. Myofibroblasts were identified in all 30 cases of malignant fibrous histiocytoma and all 4 cases of well-differentiated sclerosing liposarcoma. Though most numerous in areas of desmoplasia, in no instance did myofibroblasts constitute the dominant cellular constituent of either neoplasm. Myofibroblasts were identified with lesser frequency and in smaller numbers in fibrosarcoma, synovial sarcoma, malignant hemangiopericytoma and neuroblastoma. None were observed in a wide assortment of diverse sarcomas in which desmoplasia was not a feature. In comparison each lesion judged by light microscopy to represent either fibromatosis or fasciitis was composed principally of myofibroblasts. The demonstration of abundant myofibroblasts within a soft tissue lesion which has been subjected to wide sampling strongly suggests a benign proliferative process as opposed to a malignant neoplasm. It is hypothesized that myofibroblasts observed within collagenized regions of soft tissue sarcomas may constitute an expression of host response to neoplasia.
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PMID:Myofibroblasts in soft tissue sarcomas. 625 34

To evaluate the outcome of neonatal malignant solid tumors, we reviewed the records of 222 infants under the age of 1 year with malignant disease who were treated at the University of Texas M.D. Anderson Cancer Center over a 40-year period. Forty-five cases of neonatal (< 30 days old at the time of presentation) malignancies were found. Thirty-two infants had solid tumors and form the basis of this report. Diagnoses included soft tissue sarcoma (13), brain tumor (5), neuroblastoma (6), retinoblastoma (3), malignant melanoma (2), hemangiopericytoma (2), and nephroblastoma (1). The mean age at which initial signs and symptoms were noted was 9 days of life. Fifty-nine percent (19) presented within the first week of life, and 47% (15) presented at birth. The mean age at histological diagnosis was 54 days. The head and neck region was the most common site (18), followed by trunk (9), and extremities (5). Thirty-one patients underwent surgical resection of the primary tumor. Thirteen of those neonates received no additional chemotherapy and/or radiation therapy, whereas 18 received some combination of surgery plus perioperative chemotherapy and/or radiation therapy. Overall survival was 78% (25 of 32) with an average follow-up of 8 years (range, 2 months to 29 years). There were no survivors among those patients with distant metastatic disease at the time of diagnosis. Despite delays, prognosis is excellent in the absence of distant metastatic disease, particularly for extracranial tumors.
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PMID:Malignant solid tumors in neonates: a 40-year review. 759 29

Accumulation with bone scintigraphy using technetium-99m hydroxymethylene diphosphonate (99mTc-HMDP) in 68 cases with radiographically or pathologically verified soft tissue tumor was examined. Radiographical or histopathologic diagnoses of the 68 cases included; 14 lipomas, 11 liposarcomas, 11 neurinomas or neurofibromas, 6 malignant lymphomas, 5 malignant fibrous histiocytomas, 5 hemangioma, rhabdomyosarcomas, 2 Langerhans cell histiocytoses, 2 desmoid tumors and one each of neuroblastoma, hemangiopericytoma, angiomyxoma, plasmacytoma, liomyosarcoma, lymphangioma, fibrosarcoma, elastofibroma, synovial sarcoma, and ganglion. Thirty-seven (54%) showed positive accumulation and 31 were negative. One half of soft tissue tumors can be accumulated by 99mTc-HMDP.
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PMID:[99mTc-HMDP accumulation in soft tissue tumor]. 1119 49

As an anatomical interface between various tissues, the skull base harbors an exceptionally broad variety of neoplasms, some of which pose a major challenge for surgical pathology. The characterization of distinct immunohistochemical expression profiles and the identification of molecular genetic alterations associated with different tumor entities have significantly advanced this field. The new World Health Organization (WHO) classification of tumors of the nervous system lists 15 histopathological variants of meningioma. Of clinical importance are those entities that carry an increased risk of recurrence and a poor prognosis, i.e., the atypical meningioma (WHO grade II), clear-cell meningioma (WHO grade II), chordoid meningioma (WHO grade II), rhabdoid meningioma (WHO grade III), papillary meningioma (WHO grade III), and anaplastic meningioma (WHO grade III). Diagnostic criteria for atypical and anaplastic meningioma variants have now been stringently defined. The differential diagnosis of meningiomas includes hemangiopericytoma, hemangioblastoma, solitary fibrous tumor, sarcomas, and chordoid neoplasms. Recent data highlight the importance of distinguishing chordoma and chondrosarcoma of the skull base since chondrosarcomas show a significantly better clinical outcome. Among the less common, aggressive tumor entities in this anatomical region, infiltrating pituitary adenoma/pituitary carcinoma, superficial malignant gliomas, rhabdomyosarcoma, olfactory neuroblastoma, various sarcomas, and malignant lymphoma must be considered. Profiles of molecular genetic alterations have been established for several of these neoplasms and may facilitate the differential diagnosis. This review summarizes recent developments in the histopathological characterization, classification, and molecular pathology of neoplasms arising at the skull base.
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PMID:New developments in the pathology of skull base tumors. 1135 65

TFIIIB, TFIIIC2, and PTF/SNAPC are heteromultimeric general transcription factors (GTFs) needed for expression of genes encoding small cytoplasmic (scRNAs) and small nuclear RNAs (snRNAs). Their activity is stimulated by viral oncogenes, such as SV40 large T antigen and Adenovirus E1A, and is repressed by specific transcription factors (STFs) acting as anti-oncogenes, such as p53 and pRb. GTFs role as final targets of critical signal transduction pathways, that control cell proliferation and differentiation, and their involvement in gene expression regulation suggest that the genes encoding them are potential proto-oncogenes or anti-oncogenes or may be otherwise involved in the pathogenesis of inherited genetic diseases. To test our hypothesis through the positional candidate gene approach, we have determined the physical localization in the human genome of the 11 genes, encoding the subunits of these GTFs, and of three genes for proteins associated with TFIIIB (GTF3BAPs). Our data, obtained by chromosomal in situ hybridization, radiation hybrids and somatic cell hybrids analysis, demonstrate that these genes are present in the human genome as single copy sequences and that some cluster to the same cytogenetic band, alone or in combination with class II GTFs. Intriguingly, some of them are localized within chromosomal regions where recurrent, cytogenetically detectable mutations are seen in specific neoplasias, such as neuroblastoma, uterine leyomioma, mucoepidermoid carcinoma of the salivary glands and hemangiopericytoma, or where mutations causing inherited genetic diseases map, such as Peutz-Jeghers syndrome. Their molecular function and genomic position make these GTF genes interesting candidates for causal involvement in oncogenesis or in the pathogenesis of inherited genetic diseases.
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PMID:Genes for human general transcription initiation factors TFIIIB, TFIIIB-associated proteins, TFIIIC2 and PTF/SNAPC: functional and positional candidates for tumour predisposition or inherited genetic diseases? 1152 Nov 99

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
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PMID:Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues. 1156 28


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