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 characterisation of adrenal lesions is a common radiological dilemma. Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical. The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT. Such lesions are likely to be seen with increasing frequency given the expanding use of radiological imaging in clinical practice. Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%. Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas. This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases. We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
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PMID:Radiological localizing techniques in adrenal tumors. 1947 Dec 40

Alveolar rhabdomyosarcoma (ARMS) is an aggressive neoplasm with unique t(2;13)(q35;q14) or t(1;13)(p36;q14) chromosomal translocations, resulting in PAX3/FOXO1 and PAX7/FOXO1 fusion genes, in approximately 80% of cases. These translocations and their gene fusions have not been identified in other neoplasms, making their identification an attractive target for applying ancillary diagnostic techniques such as fluorescence in situ hybridization (FISH). We report our experience with a dual-color break-apart FISH probe for the detection of FOXO1 (13q14) rearrangements in neoplasms within the differential diagnosis of ARMS, using routinely processed formalin-fixed, paraffin-embedded tissues. A total of 52 sarcomas were analyzed including ARMS (n = 25), embryonal rhabdomyosarcomas (n = 8), neuroblastoma (n = 1), desmoplastic small round cell tumors (n = 2), Ewing sarcoma/primitive neuroectodermal tumors (EWS/PNET; n = 15), and round cell liposarcoma (n = 1). Cytogenetics and/or reverse transcription polymerase chain reaction data were available on a subset of the ARMS (n = 11) and EWS/PNET cases (n = 5). A minimum of 100 interphase tumor nuclei were evaluated for the presence of intact or translocated signals. FOXO1 gene rearrangements were identified in 88% (22/25) of ARMS (mean: 91% positive cells/case; range: 50% to 100%), whereas no rearrangements were detected in the other neoplasms examined (mean: 1.4% positive cells/case; range: 0% to 4%). FOXO1 (13q14) FISH on formalin-fixed, paraffin-embedded tissues samples showed excellent concordance with reverse transcription polymerase chain reaction and cytogenetic analyses in ARMS cases, demonstrated excellent specificity (100%) when applied to potential mimickers such as EWS/PNET, and played an important role in the differential diagnosis of small round cell tumors.
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PMID:The utility of FOXO1 fluorescence in situ hybridization (FISH) in formalin-fixed paraffin-embedded specimens in the diagnosis of alveolar rhabdomyosarcoma. 1970 58

BMS-754807 is a potent and reversible inhibitor of the insulin-like growth factor 1 receptor/insulin receptor family kinases (Ki, <2 nmol/L). It is currently in phase I development for the treatment of a variety of human cancers. BMS-754807 effectively inhibits the growth of a broad range of human tumor types in vitro, including mesenchymal (Ewing's, rhabdomyosarcoma, neuroblastoma, and liposarcoma), epithelial (breast, lung, pancreatic, colon, gastric), and hematopoietic (multiple myeloma and leukemia) tumor cell lines (IC50, 5-365 nmol/L); the compound caused apoptosis in a human rhabdomyosarcoma cell line, Rh41, as shown by an accumulation of the sub-G1 fraction, as well as by an increase in poly ADP ribose polymerase and Caspase 3 cleavage. BMS-754807 is active in vivo in multiple (epithelial, mesenchymal, and hematopoietic) xenograft tumor models with tumor growth inhibition ranging from 53% to 115% and at a minimum effective dose of as low as 6.25 mg/kg dosed orally daily. Combination studies with BMS-754807 have been done on multiple human tumor cell types and showed in vitro synergies (combination index, <1.0) when combined with cytotoxic, hormonal, and targeted agents. The combination of cetuximab and BMS-754807 in vivo, at multiple dose levels, resulted in improved clinical outcome over single agent treatment. These data show that BMS-754807 is an efficacious, orally active growth factor 1 receptor/insulin receptor family-targeted kinase inhibitor that may act in combination with a wide array of established anticancer agents.
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PMID:BMS-754807, a small molecule inhibitor of insulin-like growth factor-1R/IR. 1999 72

Ganglioside GD2 is highly expressed on neuroectoderm-derived tumors and sarcomas, including neuroblastoma, retinoblastoma, melanoma, small cell lung cancer, brain tumors, osteosarcoma, rhabdomyosarcoma, Ewing's sarcoma in children and adolescents, as well as liposarcoma, fibrosarcoma, leiomyosarcoma and other soft tissue sarcomas in adults. Since GD2 expression in normal tissues is restricted to the brain, which is inaccessible to circulating antibodies, and in selected peripheral nerves and melanocytes, it was deemed a suitable target for systemic tumor immunotherapy. Anti-GD2 antibodies have been actively tested in clinical trials for neuroblastoma for over the past two decades, with proven safety and efficacy. The main limitations have been acute pain toxicity associated with GD2 expression on peripheral nerve fibers and the inability of antibodies to treat bulky tumor. Several strategies have been developed to reduce pain toxicity, including bypassing complement activation, using blocking antibodies, or targeting of O-acetyl-GD2 derivative that is not expressed on peripheral nerves. To enhance anti-tumor efficacy, anti-GD2 monoclonal antibodies and fragments have been engineered into immunocytokines, immunotoxins, antibody drug conjugates, radiolabeled antibodies, targeted nanoparticles, T-cell engaging bispecific antibodies, and chimeric antigen receptors. The challenges of these approaches will be reviewed to build a perspective for next generation anti-GD2 therapeutics in cancer therapy.
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PMID:Engineering anti-GD2 monoclonal antibodies for cancer immunotherapy. 2429 43

Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma. For each of these tumor types, we review clinical and pathologic features, histologic clues to distinguish them from endocrine neoplasms, and recently developed immunohistochemical markers that can be particularly useful for establishing the correct diagnosis.
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PMID:Immunohistochemical Biomarkers of Mesenchymal Neoplasms in Endocrine Organs: Diagnostic Pitfalls and Recent Discoveries. 2934 Sep 97


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