Epstein-Barr virus–associated smooth muscle tumors in ataxia-telangiectasia: A case report and review
Chromosomal breakage syndromes, including ataxia-telangiectasia (AT), are autosomal recessive disorders in which DNA repair mechanisms are defective resulting in chromosomal instability. Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effe...
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| Veröffentlicht in: | Human pathology 2002-01, Vol.33 (1), p.133-136 |
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| creator | Reyes, Christine Abuzaitoun, Omar de Jong, Andrew Hanson, Celine Langston, Claire |
| description | Chromosomal breakage syndromes, including ataxia-telangiectasia (AT), are autosomal recessive disorders in which DNA repair mechanisms are defective resulting in chromosomal instability. Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important. HUM PATHOL 33:133-136. Copyright © 2002 by W.B. Saunders Company |
| doi_str_mv | 10.1053/hupa.2002.30214 |
| format | Article |
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Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important. HUM PATHOL 33:133-136. 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Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important. HUM PATHOL 33:133-136. 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Saunders Company</description><subject>Ataxia Telangiectasia - pathology</subject><subject>Ataxia Telangiectasia - virology</subject><subject>ataxia-telangiectasia</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Complex syndromes</subject><subject>Epstein-Barr virus</subject><subject>Epstein-Barr Virus Infections - complications</subject><subject>Epstein-Barr Virus Infections - pathology</subject><subject>Female</subject><subject>Herpesvirus 4, Human - isolation & purification</subject><subject>Herpesvirus 4, Human - pathogenicity</subject><subject>Humans</subject><subject>Immunocompromised Host</subject><subject>Jejunal Neoplasms - pathology</subject><subject>Jejunal Neoplasms - virology</subject><subject>Laryngeal Neoplasms - pathology</subject><subject>Laryngeal Neoplasms - virology</subject><subject>Leiomyosarcoma - pathology</subject><subject>Leiomyosarcoma - virology</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>smooth muscle tumors</subject><issn>0046-8177</issn><issn>1532-8392</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2002</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10LtuFTEQgGELEZFDoKZDbqDbE9_2YroQhYsUKU2orYk9mxjtDY83gY534A15Enw4R0qVyi7-GY0-xt5IsZWi1qd36wJbJYTaaqGkecY2staq6rRVz9lGCNNUnWzbY_aS6LsQUtamfsGOpeyUtl29YbcXC2WMU_URUuL3Ma309_cfIJp9hIyB0zjP-Y6PK_kBeV7HORGPE4cMPyNUGQeYbiP6DBThAz_jHgh5wmVOmcMUyvc-4sMrdtTDQPj68J6wb58urs-_VJdXn7-en11W3mibq9raRgZrQPpG9qh0kMrbVjQWfO9Nq0AJsEaj1Dqozte2Nfom9E0QiOg7fcLe7_cuaf6xImU3RvI4lCtxXsm10ohOa1PC033o00yUsHdLiiOkX04Kt7N1O1u3s3X_bcvE28Pq9WbE8NgfMEvw7hAAeRj6BJOP9Nhp07RtZ0tn9x0WiIKTHPmIk8cQU4F0YY5PHvEPoRuXyA</recordid><startdate>200201</startdate><enddate>200201</enddate><creator>Reyes, Christine</creator><creator>Abuzaitoun, Omar</creator><creator>de Jong, Andrew</creator><creator>Hanson, Celine</creator><creator>Langston, Claire</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200201</creationdate><title>Epstein-Barr virus–associated smooth muscle tumors in ataxia-telangiectasia: A case report and review</title><author>Reyes, Christine ; 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Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important. HUM PATHOL 33:133-136. 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| subjects | Ataxia Telangiectasia - pathology Ataxia Telangiectasia - virology ataxia-telangiectasia Biological and medical sciences Child Complex syndromes Epstein-Barr virus Epstein-Barr Virus Infections - complications Epstein-Barr Virus Infections - pathology Female Herpesvirus 4, Human - isolation & purification Herpesvirus 4, Human - pathogenicity Humans Immunocompromised Host Jejunal Neoplasms - pathology Jejunal Neoplasms - virology Laryngeal Neoplasms - pathology Laryngeal Neoplasms - virology Leiomyosarcoma - pathology Leiomyosarcoma - virology Medical genetics Medical sciences smooth muscle tumors |
| title | Epstein-Barr virus–associated smooth muscle tumors in ataxia-telangiectasia: A case report and review |
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