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Article: Malignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1

TitleMalignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1
Authors
Keywordsneural crest
neurofibromatosis
primitive neuroectodermal tumor
Issue Date1996
PublisherJohn Wiley & Sons, Inc.
Citation
Medical And Pediatric Oncology, 1996, v. 26 n. 3, p. 215-219 How to Cite?
AbstractA case of multifocal malignant peripheral neuroectodermal tumor (PNET) arising from a plexiform neurofibroma in a 4-month-old Chinese boy with neurofibromatosis type 1 (NF-1) is described. Cytogenetic culture demonstrated hypotriploid karyotype with an abnormal clone characterized by 59-60, XY, +2, +3, +6, +8, +8, +12, +i(13)(q10), +der(14)t(1;14)(q21;q32), +16, +19, +20, +mar[cp3] with no apparent abnormality of chromosome 17. The child was treated with combination chemotherapy comprising ifosphamide, vincristine and doxorubicin. Despite initial partial response the child finally died of tumor progression and pulmonary metastases 8 months after diagnosis. We believe this is the first reported case of PNET in a child with NF-1 and may support an association between these two disorders of neural crest origin.
Persistent Identifierhttp://hdl.handle.net/10722/80173
ISSN
2005 Impact Factor: 1.649
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChan, GCFen_HK
dc.contributor.authorNicholls, JMen_HK
dc.contributor.authorLee, ACWen_HK
dc.contributor.authorChan, LCen_HK
dc.contributor.authorLau, YLen_HK
dc.date.accessioned2010-09-06T08:03:15Z-
dc.date.available2010-09-06T08:03:15Z-
dc.date.issued1996en_HK
dc.identifier.citationMedical And Pediatric Oncology, 1996, v. 26 n. 3, p. 215-219en_HK
dc.identifier.issn0098-1532en_HK
dc.identifier.urihttp://hdl.handle.net/10722/80173-
dc.description.abstractA case of multifocal malignant peripheral neuroectodermal tumor (PNET) arising from a plexiform neurofibroma in a 4-month-old Chinese boy with neurofibromatosis type 1 (NF-1) is described. Cytogenetic culture demonstrated hypotriploid karyotype with an abnormal clone characterized by 59-60, XY, +2, +3, +6, +8, +8, +12, +i(13)(q10), +der(14)t(1;14)(q21;q32), +16, +19, +20, +mar[cp3] with no apparent abnormality of chromosome 17. The child was treated with combination chemotherapy comprising ifosphamide, vincristine and doxorubicin. Despite initial partial response the child finally died of tumor progression and pulmonary metastases 8 months after diagnosis. We believe this is the first reported case of PNET in a child with NF-1 and may support an association between these two disorders of neural crest origin.en_HK
dc.languageengen_HK
dc.publisherJohn Wiley & Sons, Inc.en_HK
dc.relation.ispartofMedical and Pediatric Oncologyen_HK
dc.rightsMedical and Pediatric Oncology. Copyright © John Wiley & Sons, Inc.en_HK
dc.subjectneural cresten_HK
dc.subjectneurofibromatosisen_HK
dc.subjectprimitive neuroectodermal tumoren_HK
dc.subject.meshFatal Outcomeen_HK
dc.subject.meshHumansen_HK
dc.subject.meshInfanten_HK
dc.subject.meshKaryotypingen_HK
dc.subject.meshMaleen_HK
dc.subject.meshNeoplasms, Multiple Primary - diagnosis - pathology - therapyen_HK
dc.subject.meshNeuroectodermal Tumors, Primitive, Peripheral - diagnosis - pathology - therapyen_HK
dc.subject.meshNeurofibromatosis 1 - diagnosis - pathology - therapyen_HK
dc.titleMalignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1en_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0098-1532&volume=&spage=215&epage=219&date=1996&atitle=Malignant+peripheral+neuroectodermal+tumor+in+an+infant+with+neurofibromatosis+type+1en_HK
dc.identifier.emailChan, GCF:gcfchan@hkucc.hku.hken_HK
dc.identifier.emailNicholls, JM:nicholls@pathology.hku.hken_HK
dc.identifier.emailChan, LC:chanlc@hkucc.hku.hken_HK
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.identifier.authorityNicholls, JM=rp00364en_HK
dc.identifier.authorityChan, LC=rp00373en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1002/(SICI)1096-911X(199603)26:3<215::AID-MPO12>3.0.CO;2-Fen_HK
dc.identifier.pmid8544806-
dc.identifier.scopuseid_2-s2.0-0030065939en_HK
dc.identifier.hkuros14039en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0030065939&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume26en_HK
dc.identifier.issue3en_HK
dc.identifier.spage215en_HK
dc.identifier.epage219en_HK
dc.identifier.isiWOS:A1996TT41600012-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK
dc.identifier.scopusauthoridNicholls, JM=7201463077en_HK
dc.identifier.scopusauthoridLee, ACW=7405631431en_HK
dc.identifier.scopusauthoridChan, LC=7403540707en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK

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