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dc.contributor.authorAffinita, MC
dc.contributor.authorMerks, JHM
dc.contributor.authorChisholm, JC
dc.contributor.authorHaouy, S
dc.contributor.authorRome, A
dc.contributor.authorRabusin, M
dc.contributor.authorBrennan, B
dc.contributor.authorBisogno, G
dc.coverage.spatialUnited States
dc.date.accessioned2022-12-09T13:15:23Z
dc.date.available2022-12-09T13:15:23Z
dc.date.issued2022-11-01
dc.identifierARTN e29967
dc.identifier.citationPediatric Blood and Cancer, 2022, 69 (12), pp. e29967 -en_US
dc.identifier.issn1545-5009
dc.identifier.urihttps://repository.icr.ac.uk/handle/internal/5601
dc.identifier.eissn1545-5017
dc.identifier.eissn1545-5017
dc.identifier.doi10.1002/pbc.29967
dc.description.abstractBACKGROUND: Rhabdomyosarcoma (RMS) is an aggressive malignancy, and 20% of children present with metastases at diagnosis. Patients presenting with disseminated disease very occasionally have no clear evidence of a primary tumor mass. As these patients have rarely been investigated, we report on a series of patients with RMS and unknown primary tumor site registered in the Metastatic (MTS) RMS 2008 protocol (October 2008 to December 2016) coordinated by the European pediatric Soft tissue sarcoma Study Group. METHODS: Patients were administered nine cycles of induction chemotherapy, and 48 weeks of maintenance chemotherapy. Surgery and/or radiotherapy were planned after the first assessment of tumor response, and implemented after six cycles of chemotherapy. If feasible, radiotherapy to all sites of metastasis was recommended. RESULTS: We identified 10 patients with RMS and unknown primary site, most of them adolescents (median age 15.8 years, range: 4.6-20.4). Nine had fusion-positive alveolar RMS. Multiple organ involvement was identified in seven patients, two only had bone marrow disease, and one only had leptomeningeal dissemination. All patients were given chemotherapy, four were irradiated, and none had surgery. Three patients underwent allogeneic bone marrow transplantation. At the time of this analysis, only two patients are alive in complete remission: one had received radiotherapy; and one had a bone marrow transplant. CONCLUSIONS: RMS with unknown primary tumor occurs mainly in adolescents and is typically fusion-positive alveolar. Radiotherapy may be important, but survival is poor and patients should be offered enrollment in investigational trials.
dc.formatPrint-Electronic
dc.format.extente29967 -
dc.languageeng
dc.language.isoengen_US
dc.publisherWILEYen_US
dc.relation.ispartofPediatric Blood and Cancer
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/en_US
dc.subjectbone marrow
dc.subjectrhabdomyosarcoma
dc.subjectunknown origin
dc.subjectChild
dc.subjectAdolescent
dc.subjectHumans
dc.subjectNeoplasms, Unknown Primary
dc.subjectAntineoplastic Combined Chemotherapy Protocols
dc.subjectRhabdomyosarcoma
dc.subjectRhabdomyosarcoma, Embryonal
dc.subjectRhabdomyosarcoma, Alveolar
dc.titleRhabdomyosarcoma with unknown primary tumor site: A report from European pediatric Soft tissue sarcoma Study Group (EpSSG).en_US
dc.typeJournal Article
dcterms.dateAccepted2022-08-05
dc.date.updated2022-12-09T13:14:13Z
rioxxterms.versionVoRen_US
rioxxterms.versionofrecord10.1002/pbc.29967en_US
rioxxterms.licenseref.startdate2022-11-01
rioxxterms.typeJournal Article/Reviewen_US
pubs.author-urlhttps://www.ncbi.nlm.nih.gov/pubmed/36094298
pubs.issue12
pubs.organisational-group/ICR
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Sarcoma Clinical Trials in Children and Young People
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology/Sarcoma Clinical Trials in children and young people
pubs.organisational-group/ICR/Primary Group/Royal Marsden Clinical Units
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Sarcoma Clinical Trials in Children and Young People/Sarcoma Clinical Trials in Children and Young People (hon.)
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology/Sarcoma Clinical Trials in children and young people/Sarcoma Clinical Trials in Children and Young People (hon.)
pubs.publication-statusPublished
pubs.publisher-urlhttp://dx.doi.org/10.1002/pbc.29967
pubs.volume69
dc.contributor.icrauthorChisholm, Julia
icr.provenanceDeposited by Mr Arek Surman on 2022-12-09. Deposit type is initial. No. of files: 1. Files: Pediatric Blood Cancer - 2022 - Affinita - Rhabdomyosarcoma with unknown primary tumor site A report from European.pdf


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