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Sarcoma. 2015;2015:826124. doi: 10.1155/2015/826124. Epub 2015 Nov 30.

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

Author information

1
Department of Pathology, Johns Hopkins Medicine, 600 N. Wolfe Street, Pathology B-106, Baltimore, MD 21287, USA.
2
Pediatric Cancer Biology Program, Papé Family Pediatric Research Institute, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA ; Department of Pediatrics, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA.
3
Department of Pediatrics, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA.
4
Department of Pathology, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA.
5
University of California Davis School of Medicine and Cancer Center, Sacramento, CA 95817, USA.
6
The Jackson Laboratory, 1650 Santa Ana Avenue, Sacramento, CA 95838, USA ; Champions Oncology, Hackensack, NJ 07601, USA.
7
The Jackson Laboratory, 1650 Santa Ana Avenue, Sacramento, CA 95838, USA ; Leo Universal, Inc., Torrance, CA 90505, USA.
8
The Jackson Laboratory, 1650 Santa Ana Avenue, Sacramento, CA 95838, USA.
9
Pediatric Cancer Biology Program, Papé Family Pediatric Research Institute, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA ; Department of Pediatrics, Oregon Health & Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA ; Children's Cancer Therapy Development Institute, Beaverton, OR 97005, USA.

Abstract

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.

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