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)

A series of fine-needle aspiration biopsies performed in 635 children were reviewed. The diagnoses rendered in these patients included malignant lymphoma in 139 (21.9%); Hodgkin's disease, 25 (3.9%); neuroblastoma, 58 (9.1%); Wilms' Tumor, 37 (5.8%); Ewing's sarcoma, 32 (5.0%); rhabdomyosarcoma, 25 (3.9%); retinoblastoma, 22 (3.5%); leukemia infiltrate, 33 (5.2%); and miscellaneous tumors, 52 (8.2%). In 171 patients (26.9%), the biopsy was nondiagnostic. The cytomorphological characteristics of these lesions are briefly described and illustrated. Salient morphological features are further correlated with histological and ultrastructural appearances. Immunocytochemical patterns of these tumors are also discussed briefly.
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PMID:Fine-needle aspiration biopsy of pediatric neoplasms: correlation between electron microscopy and immunocytochemistry in diagnosis and classification. 160 83

The L1 cell adhesion molecule was initially identified and characterized in mouse as a cell-surface glycoprotein that mediates neuron-neuron and neuron-Schwann cell adhesion. We have characterized L1 in humans using cDNA structural and mRNA expression analyses. We present the entire coding sequence for human L1, which predicts a 1253-amino acid protein displaying a signal sequence, transmembrane segment, RGD sequence, and potential glycosylation and phosphorylation sites. Nucleotide and deduced amino acid sequence identities between human and mouse L1 are 85% and 87%, respectively. In contrast, the amino acid identity between human L1 and the L1-related molecule chicken Ng-CAM is only 45%. Using Northern blot analyses, a single L1 transcript of 5.5 kb is detected in human fetal brain and in neuroblastoma (IMR-32) and retinoblastoma (Y-79) cell lines. L1 is also expressed in the rhabdomyosarcoma cell lines RD and A-204, which display several muscle characteristics. Two forms of L1, which differ by the presence or absence of a 12-bp cytoplasmic segment, are expressed in both human and mouse. This segment is encoded by a single exon that can be alternately spliced to give rise to the two forms, which appear to be expressed in tissue-specific patterns.
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PMID:Variants of human L1 cell adhesion molecule arise through alternate splicing of RNA. 162 59

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumour believed to arise from a pluripotential migratory neural crest cell and composed of both a mesenchymal element (most often rhabdomyosarcoma) and a neuroectodermal element (often neuroblastoma). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. We report the first case of an orbital MEM, with a review of the 17 cases previously reported from other sites.
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PMID:Primary malignant ectomesenchymoma of the orbit. 162 16

Using the avidin-biotin complex immunoperoxidase technique and antibodies to myoglobin, desmin, CLA, NSE, GFAP, keratin, fibronectin, alpha 1AT, lysozyme, S-100 protein, vimentin, cytokeratin, actin, the authors studied 60 cases of rhabdomyosarcoma (RMS) histopathologically diagnosed previously. Thirty-six cases showed both myoglobin and desmin positive stain, an objective evidence of the origin from skeletal muscles. The other 24 cases were identified as of non-skeletal muscle origin, including MFH, lymphoma, melanoma, neuroblastoma, malignant neurilemmoma, leiomyosarcoma etc. This study strongly suggests that histologic examination of RMS may lead to incorrect diagnosis. Histologically MFH and other types of spindle cell sarcomas invading normal skeletal muscles may be confused with pleomorphic RMS, lymphoma and neuroblastoma may be confused with embryonic RMS. Our findings indicate that myoglobin is a highly sensitive and specific tumor marker for RMS.
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PMID:[Immunohistochemical differential diagnosis of 60 cases of rhabdomyosarcoma]. 166 97

In order to determine the pathogenesis of fever in solid tumors, we studied the association of fever at diagnosis in children with solid tumors (malignant lymphoma, rhabdomyosarcoma, and neuroblastoma), serum levels of interleukin 1 (IL-1), and tumor necrosis factor. Thirteen of 20 patients (65%) with solid tumors were complicated with fever at diagnosis. There was no difference in C-reactive protein or IL-1 levels between the patients with and without fever, while the erythrocyte sedimentation rate and TNF levels were higher in the former than in the latter by Wilcoxon's rank sum test (p less than 0.01). These findings suggest that most febrile episodes at diagnosis in children with solid tumors are associated with the release of tumor necrosis factor.
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PMID:Tumor necrosis factor and fever at diagnosis in children with solid tumors. 169 36

A total of 2259 children with solid malignant tumors were treated at St. Jude Children's Research Hospital between the years 1962 and 1987. Of these, 112 (5%) developed spinal epidural metastasis with spinal cord compression during the course of their disease process. Metastatic epidural spinal cord compression was caused most commonly by Ewing's sarcoma and neuroblastoma, followed by osteogenic sarcoma, rhabdomyosarcoma, Hodgkin's disease, soft-tissue sarcoma, germ-cell tumor, Wilm's tumor, and (rarely) hepatoma. There was no significant difference in outcome between patients with small-cell tumors (neuroblastoma, Hodgkin's disease, and germ-cell tumors) who received only chemotherapy and/or radiation therapy and the patients with similar lesions who received a decompressive laminectomy alone or prior to chemotherapy and/or radiation therapy. Patients with spinal cord compression from metastatic sarcoma (Ewing's sarcoma, soft-tissue sarcoma, osteogenic sarcoma, and rhabdomyosarcoma) showed a significant improvement with decompressive laminectomy alone or before medical therapy, compared to those who received radiation therapy and/or chemotherapy without posterior decompression. Pediatric tumors invade the spinal canal via the neural foramen, compressing the spinal cord in a circumferential manner, allowing decompressive laminectomy (posterior approach) to be an effective surgical approach. Sixty-six percent of children who had no evidence of motor or sensory function below the level of the compression became ambulatory after surgical decompression and medical treatment, regardless of tumor type.
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PMID:Pediatric spinal epidural metastases. 184 14

Fine needle aspiration (FNA) was performed under ultrasound guidance on 17 abdominal masses in 16 pediatric patients at Baragwanath Hospital. The aspirated cellular material was assessed by conventional cytomorphology and by electron microscopy (EM). A diagnosis of malignancy was rendered for all 15 tumors that were adequately sampled (88.2%); the remaining 2 masses yielded insufficient material for either light microscopy or EM. Cytologic cell typing (including the use of EM) was successful in 12 of the 15 tumors (80%) as compared with the histologic diagnosis. EM was in agreement with the initial cytologic diagnosis in eight tumors, but corrected the initial impression in four tumors. The tumors with adequate aspirates included nine nephroblastomas and single examples of neuroblastoma, hepatoblastoma, non-Hodgkin's lymphoma, rhabdomyosarcoma, renal carcinoma and malignant rhabdoid tumor. The last three were not accurately typed by cytology plus EM. These preliminary results suggest that FNA cytology with adjunctive EM could become a useful technique in the preoperative assessment of pediatric abdominal tumors.
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PMID:Fine needle aspiration of pediatric abdominal masses. Cytologic and electron microscopic diagnosis. 185 56

In the magnetic resonance (MR) imaging examinations of three children with tumors (two neuroblastoma, one rhabdomyosarcoma) and three with leukemia, the marrow demonstrated a diffuse, uniform pattern of hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The authors observed that this reversal ("flip-flop") of the usual MR characteristics of fatty marrow was seen in the epiphyses, metaphyses, and diaphyses. The purpose of this study was to establish the radiographic and clinicopathologic correlates of this MR finding on the basis of findings from plain radiographs, bone scans, and bone marrow aspirates. Plain radiographs and bone scans demonstrated either normal findings or changes limited to the metaphyses. In all patients, analysis of bone marrow aspirates demonstrated metastases. The authors concluded that even in the absence of evidence of discrete bone metastases on a plain radiograph or a bone scan, this diffuse and uniform "flip-flop" pattern reflects diffuse marrow replacement by tumor cells.
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PMID:MR imaging of diffuse bone marrow replacement in pediatric patients with cancer. 192 9

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


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