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A Patient-Derived Xenograft Model of Parameningeal Embryonal Rhabdomyosarcoma for Preclinical Studies.

Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children and adolescents. Parameningeal eRMS is a variant that is often more difficult to treat than eRMS occurring at other sites. A 14-year-old female with persistent headaches and rapid weight loss was diagnosed w...

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Published in:Sarcoma pp. 1 - 8
Main Authors: Hooper, Jody E., Cantor, Emma L., Ehlen, Macgregor S., Banerjee, Avirup, Malempati, Suman, Stenzel, Peter, Woltjer, Randy L., Gandour-Edwards, Regina, Goodwin, Neal C., Yang, Yan, Kaur, Pali, Bult, Carol J., Airhart, Susan D., Keller, Charles
Format: case study diagnostic images pictorial research tables/charts Journal Article
Published: Wiley-Blackwell 11/30/2015
Online Access:View this record in EBSCOhost
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        1357714X
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      dt: 11/30/2015
      pid: 480
      pub: Wiley-Blackwell
      place: Malden, Massachusetts
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        113627947
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        10.1155/2015/826124
        113627947
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        atl: A Patient-Derived Xenograft Model of Parameningeal Embryonal Rhabdomyosarcoma for Preclinical Studies.
      aug:
        au:
          Hooper, Jody E.
          Cantor, Emma L.
          Ehlen, Macgregor S.
          Banerjee, Avirup
          Malempati, Suman
          Stenzel, Peter
          Woltjer, Randy L.
          Gandour-Edwards, Regina
          Goodwin, Neal C.
          Yang, Yan
          Kaur, Pali
          Bult, Carol J.
          Airhart, Susan D.
          Keller, Charles
        affil: Department of Pathology, Johns Hopkins Medicine, 600 N. Wolfe Street, Pathology B-106, Baltimore, MD 21287, USA
      sug:
        subj:
          Sequence Analysis
          Rhabdomyosarcoma
          Weight Loss
          Models, Biological
          Dactinomycin
          Neoplasms
          Female
          Child
          Log-Rank Test
          Data Analysis
          Adolescence
          Prospective Studies
          Case Studies
          Child: 6-12 years
          Adolescent: 13-18 years
          Female
      ab: Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children and adolescents. Parameningeal eRMS is a variant that is often more difficult to treat than eRMS occurring at other sites. A 14-year-old female with persistent headaches and rapid weight loss was diagnosed with parameningeal eRMS. She progressed and died despite chemotherapy with vincristine, actinomycin-D, and cyclophosphamide plus 50.4 Gy radiation therapy to the primary tumor site. Tumor specimens were acquired by rapid autopsy and tumor tissue was transplanted into immunodeficient mice to create a patient-derived xenograft (PDX) animal model. As autopsy specimens had an ALK R1181C mutation, PDX tumor bearing animals were treated with the pan-kinase inhibitor lestaurtinib but demonstrated no decrease in tumor growth, suggesting that single agent kinase inhibitor therapy may be insufficient in similar cases. This unique parameningeal eRMS PDX model is publicly available for preclinical study.
      pubtype: Academic Journal
      doctype:
        case study
        diagnostic images
        pictorial
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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