A Patient-Derived Xenograft Model of Parameningeal Embryonal Rhabdomyosarcoma for Preclinical Studies.
Document Type
Article
Publication Date
2015
JAX Source
Sarcoma 2015; 2015:826124.
Volume
2015
First Page
826124
Last Page
826124
ISSN
1357-714X
PMID
26696773
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. Sarcoma 2015; 2015:826124.
Recommended Citation
Hooper J,
Cantor E,
Ehlen M,
Banerjee A,
Malempati S,
Stenzel P,
Woltjer R,
Gandour-Edwards R,
Goodwin N,
Yang Y,
Kaur P,
Bult C,
Airhart S,
Keller C.
A Patient-Derived Xenograft Model of Parameningeal Embryonal Rhabdomyosarcoma for Preclinical Studies. Sarcoma 2015; 2015:826124.